There is no doubt, they are not meeting the diagnostic criteria of Kawasaki disease (fever of several days of evolution, exanthema, conjunctival injection, red lips, raspberry tongue, edema of acral parts and adenopathy).

A 4-year-old girl presenting with a high fever of 6 days' evolution. On clinical examination she presents an erythematous maculopapular rash on the trunk and genital area, with a tendency to confluence, without becoming scarlatiniform; conjunctival injection without secretions and red lips with raspberry tongue. She also presents erythema with edema in hands and feet and a unilateral cervical adenopathy of 2 cm in diameter. The most likely clinical diagnosis of suspicion is:

Catastrophic Kawasaki disease unresponsive to IVIG in a 3-month-old infant: a diagnostic and therapeutic challenge.

The present report describes the severe evolution of Kawasaki disease in a three-month-old infant. The ailment was initially atypical in its presentation, with the patient exhibiting only persistent fever in association with a progressive lethargy and maculopapular rash on the face, trunk and limbs erroneously diagnosed as roseola infantum. On the 10th day of the condition, mainly due to the unexplained persistence of fever, the infant was admitted to a local hospital. The typical features of KD appeared only on the 14th day of illness with the relapse of the maculopapular rash in association with non-purulent conjunctivitis; dry, reddish and fissured lips; tongue with reddish and hypertrophic papillae; erythema and edema of the palms and soles. During the following days, the ailment rapidly evolved to a catastrophic clinical picture characterized by generalized vasculitis, splenic infarction, pulmonary thrombosis, giant right and left coronary aneurysms, dilatation of common and internal iliac arteries and progressive ischemia of the distal third of the feet resulting in necrotic lesions of both halluces. Appropriate therapy was initiated, but repeated administration of intravenous immunoglobulin G (IVIG) followed by three days of administration of methylprednisolone did not abate the intense inflammatory activity. The remission of inflammation and regression of vascular lesions were only achieved during the following five weeks after the introduction of methotrexate associated with etanercept. The report of this case aims to draw attention to severe forms of KD that exhibit an unfavorable evolution and can be extremely refractory to the conventional therapy.

Late Treatment and Recurrence of Kawasaki Disease in a Moroccan Infant.

While the diagnosis of typical form of Kawasaki disease (KD) is obvious, this multifaceted disease continues to surprise us. We report the case of a recurrent Kawasaki disease in an infant. At the age of 13 months, the infant was diagnosed with complete Kawasaki disease; he presented with prolonged fever, bilateral conjunctivitis, enanthem, exanthema, edema of the lower limb, peeling, and biological inflammatory syndrome. He was treated with intravenous immunoglobulin (IVIG) associated with a high dose of aspirin and then an antiplatelet dose with a good clinical-biological evolution. The echocardiography was normal. Seven months later, the patient was again admitted, in a similar picture: a prolonged fever evolving for 7 days, bilateral conjunctivitis, enanthem, cervical adenopathy of 1.5 cm/1 cm, scarlatiniform erythema, pruriginous of the trunk and limb, and peeling of the toes, with indurated edema of the hands and feet. The rest of the examination was normal except the irritability. The diagnosis of recurrent KD was made according the five criteria of the American Heart Association. The echocardiography was normal again. The infant received IVIG with good outcome. Despite its rarity, the possibility of recurrence of KD should be known by clinicians, so as not to delay the specific management of vasculitis whose stakes in terms of prevention of coronary artery lesions are well known. Our case confirms the possibility of this recurrence.

InternalMed_Harrison

The cutaneous eruption in Kawasaki disease (Chap. 385) is polymorphous, but the two most common forms are morbilliform and scarlatiniform. Additional mucocutaneous findings include bilateral conjunctival injection; erythema and edema of the hands and feet followed by desquamation; and diffuse erythema of the oropharynx, red strawberry tongue, and dry fissured lips. This clinical picture can resemble TSS and scarlet fever, but clues to the diagnosis of Kawasaki disease are cervical lymphadenopathy, cheilitis, and thrombocytosis. The most serious associated systemic finding in this disease is coronary aneurysms secondary to arteritis. Scarlatiniform eruptions are also seen in the early phase of SSSS (see “Vesicles/Bullae,” above), in young adults with Arcanobacterium haemolyticum infection, and as reactions to drugs.

[Acute renal insufficiency in Kawasaki disease].

Kawasaki disease is an acute inflammatory condition characterized by various combinations of features but renal involvement is rare. This report is of a case of Kawasaki disease complicated by acute kidney failure. A 10 year-old girl was admitted because of acute renal failure with fever. She developed a high fever, and her general condition was poor; she had developed a macular erythematous rash 10 days earlier for which she was given cefadroxil. At admission, she remained febrile and had strawberry tongue, pharyngitis, dry erythematous lips, bilateral conjunctivitis, cervical lymphadenopathy and desquamation of the skin on her hands. She was anemic (hemoglobin = 9.6 g%), leukocytotic (33,100/mm3), but with no burr, fragmented red blood cells or thrombocytopenia. Her plasma C-reactive protein level was 236 mg/l; her blood urea was 9.5 mmol/l, her creatininemia 288 mumol/l and proteinuria was 0.5 g/l without hematuria. Urine cultures did not grow. Her blood transaminase and gammaglutamyltransferase activities were elevated. Ultrasonography of kidneys and coronary arteries was normal. Kidney biopsy performed one day after admission showed no vascular or glomerular changes, but renal tubular necrosis, indicating urinary excretion of pigments. Tests for myoglobinemia, myoglobinuria and blood muscle enzyme activities were all positive. The renal failure disappeared within 10 days but the fever and inflammatory manifestations persisted for 1 1/2-2 months despite two treatments of intravenous gammaglobulins and continuous salicylate administration. The patient developed arthralgias at the end of the first month of disease, but recovered without renal or vascular complications. Several cases of renal involvement have been reported during the course of Kawasaki disease. They have been rarely documented by histological examination so that the vascular origin of changes has not been demonstrated. Myoglobinuria, as seen in muscular crush injury, and in our case possibly due to malignant hyperthermia, may be responsible for the transient acute renal failure.

Kawasaki Disease -- History and Physical

In 2014, the American Heart Association (AHA) published the criteria needed to establish a diagnosis. [1] However, it is important to note that children who fall short of the full criteria but have cardiac abnormalities on echocardiogram meet the diagnosis of KD. [1] The patient must have fevers for five or more days, with at least four of the following criteria (either all at once or over a series of days): Bilateral painless bulbar conjunctival injection without exudate Erythematous mouth and pharynx, strawberry tongue or red, cracked lips Polymorphous exanthem (morbilliform, maculopapular, or scarlatiniform) Swelling of hands and feet with erythema of the palms and soles Cervical lymphadenopathy (over 1.5 cm in diameter)

An unusual pattern of arthritis in a child with Kawasaki syndrome.

Arthritis is reported in one-third of cases with Kawasaki syndrome. It may have an early or a late onset form. We present a 15-month-old-girl who had been referred with complaints of pain and swelling in her left shoulder. Physical examination revealed bulbar conjunctival injection, erythematous lips and pharynx, strawberry tongue, erythematous rash, edema and erythema of the left shoulder, left knee, right elbow and right wrist, and moderate distress in the left shoulder and left hip. She was diagnosed with Kawasaki syndrome, and intravenous immunoglobulin infusion (IVIG) 2 g/kg and aspirin (100 mg/kg/day) were instituted. The patient had two additional episodes of arthritis involving the hip joint on the 8th day, and the shoulder and metacarpophalangeal (MCP) and interphalangeal (IP) joints of her right hand on the 15th day. Turbid material was aspirated in both instances; Gram and Wright's staining of this material showed many leukocytes but no bacteria. A second dose of IVIG (1 g/kg) was given. At the end of the third week all extremities were painless, with a normal range of motion. Arthritis in our patient was the presenting sign, having a 'septic arthritis mimicking' and 'biphasic' pattern. Although the patient presented with severe and recurrent arthritis, which is significantly correlated with severe multisystem disease and the presence or development of coronary artery aneurysm, the response to IVIG was excellent.

A Case of Kawasaki Disease with Intussusception.

Kawasaki disease (KD) is a systemic vasculitis of unknown cause and is associated with various digestive disorders, although only a few cases of intussusception associated with KD have been reported. We describe a case of intussusception followed by KD in a 3-year-old boy. The patient was admitted to our hospital for evaluation of severe abdominal pain. Because the target sign was seen on ultrasonography, intussusception was diagnosed and hydrostatic reduction was performed. On the second day after admission, he developed a high fever (38°C) and an irregular rash over his whole body. On the fourth day after admission, the high fever continued, and bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, strawberry tongue, indurated edema of the dorsa of the hands and feet, and diffuse erythema of the palms and soles appeared, and KD was ultimately diagnosed. He was treated with intravenous immunoglobulin 2 g/kg, aspirin 30 mg/kg/day, and prednisolone 2 mg/kg/day. The high fever and other clinical symptoms resolved immediately after the start of treatment. There was no relapse of KD symptoms after initial treatment, and periungual desquamation was observed on the 10th day after admission. He was discharged on the 15th day, without abnormalities such as coronary dilatation, 3 months after the onset of KD symptoms. Patients with intussusception and KD were older (≥3 years vs <3 years) than those with intussusception alone. In addition, the site of intussusception in KD was mainly colonic rather than ileocolic. If intussusception precedes development of the characteristic clinical symptoms of KD, diagnosis of KD may be delayed. KD should be considered in children older than 3 years with intussusception at a colonic site.

Mediastinal lymphadenopathy: a variant of incomplete Kawasaki disease.

A 14-month-old girl presented with a 4-d history of fever and generalized exanthema. Four characteristic symptoms of incomplete Kawasaki disease (KD) were present on admission (fever, rash, non-purulent conjunctival injection, oropharyngeal changes) and then followed by oedema of the hands and feet and mild plantar desquamation. The typical laboratory features of KD, such as elevated erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and positive C-reactive protein were also seen. Ultrasound examination of the mediastinum revealed the presence of a lymph node, 30 mm in diameter, below the tracheal carina. Thoracic CT scan confirmed the mediastinal lymph node. The patient was treated with aspirin and intravenous gamma-globulin. Ultrasound study of the mediastinum, which was carried out 6 weeks after hospital discharge, showed that the lymph node had disappeared. This case illustrates that lymph nodes other than cervical lymphadenopathy should be sought when the diagnosis of classical or atypical KD is suspected.

Unusual manifestations of Kawasaki disease with retropharyngeal edema and shock syndrome in a Taiwanese child.

We report a 3-year-old girl with Kawasaki disease who presented with retropharyngeal edema and shock syndrome. This is the first reported case in Taiwan. The patient initially presented with fever, cough, and pyuria followed by rapidly progressive enlarged bilateral cervical lymphadenopathy. On the third day of the fever, computed tomography for airway compression sign found widening of the retropharyngeal space mimicking a retropharyngeal abscess. Later, an endotracheal tube was inserted for respiratory distress. A skin rash over her trunk was also noted. On the fifth day of the fever, the clinical course progressed to hypotension and shock syndrome. Because of more swelling of bilateral neck lymph nodes, computed tomography was arranged again and revealed partial resolution of the edematous changes in the retropharyngeal space. Edema of the hands and feet, bilateral bulbar conjunctivitis, and fissured lips were subsequently found. The diagnosis of Kawasaki disease was confirmed on the eighth day of fever. There was no evidence of bacterial infection. She was administered intravenous immunoglobulin (2 mg/kg) and high dose aspirin (100 mg/kg/day). One day later, the fever subsided, and her blood pressure gradually became stable. Heart echocardiography on the Day 13 revealed dilated left coronary artery and mitral regurgitation. Follow-up echocardiography six months later showed normal coronary arteries. To date, the patient has not experienced any complications. This case illustrates that retropharyngeal edema and shock syndrome can be present in the same clinical course of Kawasaki disease. Clinicians and those who work in intensive care units should be aware of unusual presentations of Kawasaki disease to decrease rates of cardiovascular complications.

[A case report of acute Q fever showing Kawasaki disease-like symptoms in a 9-year-old girl].

A 9-year-old girl developing fever and hyperemia of both bulbar conjunctiva 5 days before admission to the Saitama Children's Medical Center after antibiotics proved ineffective was found on admission to have general fatigue and a temperature of 39 degrees C. Physical examination showed hyperemia of the bulbar conjunctiva, fissures of the lips, redness of the pharynx, and swelling of the cervical lymph nodes. Laboratory tests detected neutrophilia (11,200/microL), mild anemia (11.4g/dL), thrombocytopenia (110,000/microL), and elevated serum aspartate aminotransferase (242IU/L), alanine aminotransferase (328IU/L), and C-rective protein (25.2 mg/dL). Autoantibodies such as anti-nuclear, anti-SS-A/Ro, and anti-Jo-1 were also found. Echocardiography showed no abnormality of the coronary arteries. She was diagnosed as having incomplete Kawasaki disease on day 7 of illness, necessitating that a high dose of immunoglobulin be given intravenously. Her temperature dropped temporarily to 37 degrees C, but she developed erythema of the cheek and fever. Intravenous immunoglobulin was restarted, and minocycline introduced because her daily contact with a pet cat indicated richettsial infection such as Q fever. Mild fever, muscle pain, and elevated C-reactive protein did not improve, but clinical signs and symptoms gradually lessened after ibuprofen was given, then disappeared. A definitive diagnosis of Q fever was made through an over 4-fold rise in phase II IgG antibody titers against Coxiella burnetii, titer of less than 1 : 16 on day 14 of illness, and titer of 1 : 256 on day 34. This case study describes on atypical case of Q fever with clinical manifestations mimicking Kawasaki disease.

Uncommon erythema multiforme in small children: experience of a single Romanian pediatric unit: Two case reports.

Erythema multiforme (EM) is an immune-mediated disease with mucocutaneous localization and plurietiologic determinism. The term "multiforme" refers to the variety of aspects that the lesions can take from patient to patient and during evolution in a single patient. We have selected 2 cases of small children diagnosed with different etiology of EM to illustrate the importance of a correct and fast diagnosis. Case 1 involves a 2-year-old girl from a rural area who presented with fever and pruritic erythematous papular eruption. The onset of the symptoms was 3 days before presentation with fever and ulcerative lesions on the oral and labial mucosa, followed by the appearance of erythematous macular lesions, with progressive confluence to intense pruritic patches. The 2nd involves a 2-year-old boy with fever, loss of appetite, productive cough, and petechiae. He had corticosensible immune thrombocytopenia from the age of 6 months, with many recurrences. The patient received treatment with ampicillin/sulbactam and symptomatics for an erythemato-pultaceous angina. During the 2nd day of treatment the patient developed an erythematous macular eruption on the face, scalp, trunk, and limbs, with bullae formation. The 1st patient was diagnosed based on biologic findings: positive inflammatory syndrome, elevated level of anti-Mycoplasma pneumoniae immunoglobulin M antibodies and immunoglobulin E. Histopathologic examination described papillary dermal edema, inflammatory infiltrate, and lymphocyte exocytosis. In the 2nd case, the hemoleucogram identified 12,000/mm platelets and the medulogram aspect was normal. Serology for Epstein-Barr virus was negative. The diagnosis was EM secondary to M pneumoniae infection in case 1 and secondary to administration of ampicillin/sulbactam in case 2. In both cases, etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines was administered. Because of specific etiology, the 1st case received antibiotics. The evolution was favorable in 10 to 14 days; the patients were discharged after etiopathogenic treatment consisting of steroidal antiinflammatory drugs, antihistamines, and/or antibiotics. Performing a detailed clinical examination, medical history of drug use, infection or general diseases can establish a good diagnosis of EM. Histopathologic examination can help. The treatment is etiologic, pathogenic, and symptomatic. EM usually has a self-limited evolution.

Concomitant dengue infection and Kawasaki disease in an infant: a case report and literature review.

A previously healthy 11-month-old girl presented with fever and rash for 6 days. Physical examination revealed an irritable infant with a high fever, injected conjunctivae, red cracked lips, posterior auricular lymphadenopathy, hepatomegaly, generalized erythematous maculopapular rash and petechial hemorrhage on trunk, face and extremities. Complete blood count showed atypical lymphocytosis and thrombocytopenia. Dengue infection was initially diagnosed. The persistent fever and clinical manifestations of Kawasaki disease (KD) were observed on day 8 with high erythrocyte sedimentation rate (56 mm/hr). Treatment of KD included intravenous immunoglobulin on day 9 of the illness. Desquamation of the fingers was found on day 15 of the illness. Ectasia of left coronary artery with small aneurysmal dilatation was detected by echocardiography on day 15 of the illness. Hemagglutination-inhibition test and enzyme-linked immunosorbent assay for dengue virus eventually showed a four-fold rising. According to the literature review, this is the second reported case of dengue infection concomitant with KD. The natural course of each disease may be modified and causes some difficulties in diagnosis and management.

First_Aid_Step2

Cervical lymphadenopathy (often painful and unilateral, with at least one node > 1.5 cm). Diffuse mucous membrane erythema (e.g., “strawberry tongue”); dry, red, chapped lips. Erythema of the palms and soles; indurative edema of the hands and feet; late desquamation of the fingertips (in the subacute phase). Other manifestations include sterile pyuria, gallbladder hydrops, hepatitis, and arthritis. Untreated Kawasaki disease can lead to coronary aneurysms and even myocardial infarction! Conjunctivitis Rash Adenopathy Strawberry tongue Hands and feet (red, swollen, f aky skin) BURN (fever > 40°C for ≥ 5 days) Subacute phase: Begins after the abatement of fever and typically lasts for an additional 2–3 weeks. Manifestations are thrombocytosis and elevated ESR. Untreated children may begin to develop coronary artery aneurysms (40%); all patients should be assessed by echocardiography at diagnosis.

Nontypical presentation of a common disease: a case report.

Kawasaki disease is an idiopathic medium-sized vasculitis that occurs primarily in infants and children younger than 5 years of age. Atypical Kawasaki disease applies to patients who do not fulfill the complete criteria of fever of 5 days or more with at least four of five features: bilateral conjunctival injection, changes in the lips and oral cavity, cervical lymphadenopathy, extremity changes, and polymorphous rash. Acute kidney injury is defined as a sudden decline in kidney function within hours, including structural injuries and loss of function. Acute kidney injury is extremely common in hospitalized pediatric patients. However, it is rarely documented in Kawasaki disease. Acute kidney injury is underestimated in Kawasaki disease due to the lack of a clear definition of age-specific normal serum creatinine levels and routine renal functions. This report describes a case who presented with clinical features suggestive of atypical Kawasaki disease and developed acute kidney injury. A 2-year-old Saudi girl had a history of high-grade fever for 5 days, moderate dehydration, dry cracked lips, poor appetite, and generalized erythematous rash; therefore, she was diagnosed to have incomplete Kawasaki disease. Laboratory investigations revealed normochromic normocytic anemia, leukocytosis, thrombocytosis, high inflammatory markers, and acute kidney injury stage III. An echocardiogram showed a 4-mm dilatation on the left main coronary artery and a 3-mm dilatation on the right. A renal biopsy was not performed to identify the cause of the injury as it showed improvements after the start of the specific therapy for Kawasaki disease; intravenous immune globulin at a dose of 2 g/kg, aspirin at a high dosage of 80 mg/kg/day, and prednisolone at 2 mg/kg. In addition to the acute kidney injury management, normal saline boluses were followed by furosemide at a 2 mg/kg dose. Her urine output increased, and her renal functions normalized. She was discharged in good condition after 10 days. It is valuable to check renal function tests in a confirmed case of Kawasaki disease to reduce the negative consequences of late acute kidney injury discovery. Early detection and intervention make a substantial difference in acute kidney injury management.

Rubeola (Measles) -- Differential Diagnosis

Measles should be distinguished from similar presenting exanthemic diseases of childhood, autoimmune processes, and adverse drug reactions. Rubella causes a rash similar to measles with head to caudal distribution, mild respiratory symptoms, the absence of conjunctivitis. Still, it is accompanied by the presence of adenopathies - which is characteristic of this disease. Roseola is characterized by an illness beginning with a high fever, which subsides after a few days, accompanied by the appearance of a rash in the central part of the body, without the presence of Koplik's points. Mononucleosis is a febrile viral disease, a characteristic course with few symptoms during childhood, contrary to what happens in more advanced ages.  Mononucleosis manifests itself by pharyngeal compromise, polyadenopathy, and hepatosplenomegaly, and the rash can have different forms of presentation. In Kawasaki disease, there is an ocular compromise with the presence of conjunctivitis without exudate, and the respiratory compromise is not part of this pathology. Group A Streptococcus (particularly Scarlet fever) may present with a similar rash (a coarse, sandpaper-like, blanching, erythematous) to measles in association with pharyngitis. [5] [14]

Kawasaki disease.

Kawasaki disease (KD) is a common vasculitic disorder usually seen in children below 5 years of age. The disease can present with protean clinical manifestations which include high grade fever (for at least 5 days), rash, redness of the lips and a typical strawberry tongue, cervical lymph node enlargement (often unilateral), swelling over the hands/feet and, later a characteristic peripheral desquamation over the fingers and toes. These clinical features appear sequentially and the findings may change from day-to-day. Thus, all these features may not be seen together at any one point of time. The diagnosis rests on the recognition of this characteristic temporal sequence of clinical events, none of which are, by themselves, pathognomonic. Establishing a diagnosis of KD may be further complicated by the occurrence of several other, seemingly unrelated, clinical features. These include irritability, neck stiffness, sterile pyuria, pneumonitis, hydrops of the gallbladder and hepatitis among many others. There is no laboratory test that can help in confirming a diagnosis of KD. Left untreated, up to 20% of children with KD can develop coronary aneurysms with catastrophic long term sequelae. It is important to diagnose KD in the first 10 days of the illness so that appropriate therapy with intravenous immunoglobulin and aspirin can be Initiated. All paediatricians, and physicians looking after children, need to be aware of this condition which is now being increasingly recognized in India.

Kawasaki syndrome.

Kawasaki syndrome, also known as mucocutaneous lymph node syndrome, is an acute vasculitis of infants and young children. We describe a four-year-old girl who presented with fever, a diffuse erythematous maculopapular rash, bilateral nonpurulent bulbar conjunctivitis, dry, red, fissured lips, a tongue with a strawberry "appearance", an erythematous pharynx, indurative erythema, and edema and desquamation of the face, hands and feet. She probably developed mitral valve prolapse during the course of the disease. The diagnosis of Kawasaki syndrome was arrived at by excluding other diseases and by the presence of all the clinical criteria for Kawasaki syndrome. Since this syndrome is rarely encountered in Turkey, this case is presented and the literature regarding the syndrome is reviewed.

Kawasaki disease

Classically, five days of fever plus four of five diagnostic criteria must be met to establish the diagnosis. The criteria are: erythema of the lips or oral cavity or cracking of the lips rash on the trunk swelling or erythema of the hands or feet red eyes (conjunctival injection) swollen lymph node in the neck of at least 15 mm Many children, especially infants, eventually diagnosed with Kawasaki disease, do not exhibit all of the above criteria. In fact, many experts now recommend treating for Kawasaki disease even if only three days of fever have passed and at least three diagnostic criteria are present, especially if other tests reveal abnormalities consistent with Kawasaki disease. In addition, the diagnosis can be made purely by the detection of coronary artery aneurysms in the proper clinical setting. Investigations A physical examination will demonstrate many of the features listed above.

Kawasaki disease in healthy young adult.

This report describes a 26-year-old woman who fulfills the criteria for the diagnosis of Kawasaki disease or mucocutaneous lymph node syndrome, an acute febrile illness that usually afflicts young children. The diagnosis is made in persons with fever lasting 5 or more days when four of the following criteria are met: bilateral injection of ocular conjunctivae; the involvement of the mucous membranes of the upper respiratory tract consisting of any combination of the following--redness and fissuring of lips; "strawberry tongue," or erythema of the pharynx; involvement of the peripheral extremities characterized in the early stages by an indurative erythematous rash of palms and soles followed by membranous desquamation; polymorphous nonvesicular truncal exanthem; and acute nonsuppurative enlargement of cervical lymph nodes. An added stipulation is that the illness must not be attributable to a known disease process.

Kawasaki disease in a 4-year-old boy.

A 4-year-old boy experienced sudden fever, followed by a rash on the trunk and extremities and erythema of the pharynx. Five days later, the fever remained and erythema appeared on the oropharynx, tongue, and lips. The skin of the palms and soles became erythematous and indurated, and both conjunctivae became injected. Desquamation of the skin occurred on both thumbs and one finger, and an anterior cervical lymph node was found to be enlarged. The patient was diagnosed as having Kawasaki disease, and treatment with aspirin was started. The desquamation progressed to involve the entire surface of the palms and soles, and then symptoms resolved. Twenty years after recognition of Kawasaki disease, this enigmatic illness continues to defy attempts to understand its etiology and pathogenesis. Most experts agree that the cause is either an environmental toxin or an infectious agent, but other possible causative agents may need to be proposed and investigated.

Twenty-year-old woman presenting with typical Kawasaki disease.

We describe adult-onset Kawasaki disease (KD) and review clinical manifestations and treatment guidelines. Our patient is a 20-year-old female who initially presented to an outside hospital for fever, cervical lymphadenopathy, malaise, exudative tonsillitis, and skin eruption. She received antibiotics for suspected exudative pharyngitis, but experienced continued fevers and presented to the UCLA emergency room one week later. She had diffuse petechial macules coalescing into reticulated patches, fingertip peeling, conjunctival injection, oral erosions, and tongue swelling. Despite her age, given her constellation of symptoms, a diagnosis of typical KD was favored. She was started on high dose aspirin and IVIG, with improvement of rash and conjunctivitis. She was discharged on 325mg of aspirin daily with close follow-up. This case highlights the challenge of diagnosing KD in adults. Although this patient had classic symptoms, she was likely misdiagnosed because KD is rare in adults and without validated criteria. Our patient met the pediatric criteria, suggesting these should be considered when clinical suspicion for adult-onset KD is high. Adult-onset KD is most commonly misdiagnosed as toxic shock syndrome or drug-induced hypersensitivity syndrome and these are important to rule-out. Treatment with high-dose aspirin and IVIG is well established and should be initiated promptly.

An adult case of kawasaki disease in a pregnant Japanese woman: a case report.

Kawasaki disease is an acute febrile disease predominantly seen in young children. We report a case of Kawasaki disease in a 32-year-old pregnant woman. She developed a generalized erythematous skin rash accompanied by high fever. Bilateral conjunctival congestion, tender cervical lymphadenopathy, an edematous lower lip and peripheral edema followed by desquamation were observed. She was successfully treated with aspirin and intravenous gammaglobulin (1 g/kg/day). Her course was not complicated by coronary artery aneurysm and she delivered a healthy baby. To the best of our knowledge, this is the first case of Kawasaki disease in a pregnant woman. We suggest that Kawasaki disease should be included in the differential diagnosis of a generalized, erythematous skin rash accompanied by high fever in adults.

Systemic vasculitis

Kawasaki disease. Usually in children (age<4), it affects large, medium, and small vessels, prominently the coronary arteries. Associated with a mucocutaneous lymph node syndrome. Diagnosis requires fever lasting five days or more with at least four out of five criteria: Bilateral conjunctival injection Injected or fissured lips, injected pharynx, or strawberry tongue Erythema of palms/soles, edema of hands/feet, periungual desquamation Polymorphous rash Cervical lymphadenopathy (at least one node > 1.5 cm) Isolated cerebral vasculitis. Affects medium and small arteries over a diffuse CNS area, without symptomatic extracranial vessel involvement. Patients have CNS symptoms as well as cerebral vasculitis by angiography and leptomeningeal biopsy. Small vessel vasculitis There are several vasculitides that affect small vessels.

InternalMed_Harrison

in recent years, although strains of GAS that produce pyrogenic exotoxins continue to be prevalent in the population. The symptoms of scarlet fever are the same as those of pharyngitis alone. The rash typically begins on the first or second day of illness over the upper trunk, spreading to involve the extremities but sparing the palms and soles. The rash is made up of minute papules, giving a characteristic “sandpaper” feel to the skin. Associated findings include circumoral pallor, “strawberry tongue” (enlarged papillae on a coated tongue, which later may become denuded), and accentuation of the rash in skinfolds (Pastia’s lines). Subsidence of the rash in 6–9 days is followed after several days by desquamation of the palms and soles. The differential diagnosis of scarlet fever includes other causes of fever and generalized rash, such as measles and other viral exanthems, Kawasaki disease, TSS, and systemic allergic reactions (e.g., drug eruptions).

[Kawasaki disease complicated with hemorrhagic enteritis mimicking intestinal obstruction: report of one case].

A 6-month-old little boy presented with fever and cervical lymphadenopathy for four days. On admission, he was found to have conjunctival congestion of both eyes, reddened and fissured lips, straw-berry tongue, macular rash at the trunk and erythematous change on the BCG injection site. The diagnosis of Kawasaki disease was made and aspirin therapy (100 mg/kg/day) was started. On the 3rd hospital day, he developed abdominal distention, jaundice, poor activity and tachypnea. Bowel sound was silent on auscultation. Nasogastric tube was inserted for decompression and bilious material was drained out continuously. In addition, bloody, mucoid stool passage was noted. An abdominal radiography revealed dilatation of the small bowel and paucity of the colon gases. On the abdominal ultrasound, hydrops of gallbladder, marked ascites and silent and dilated bowel loops were found. Despite of supportive care, abdominal symptoms persisted and condition deteriorated. Laparotomy was done on the following day. At operation, it was found that the patient developed severe petechia on the long segment of small intestine from 15cm distal to the Treitz ligament to ileocecal valve and some fibrin plagues on the terminal ileum at 25cm proximal to the ileocecal valve were also found.

14-month-old girl with prolonged fever, a desquamative rash, and a new left hemiplegia.

A young child with fever, erythematous rash, and conjunctivitis in sub-Saharan Africa is usually a case of measles. We report a-14-month-old girl with a prolonged fever, a desquamating erythematous rash, and a new left hemiplegia. This was initially diagnosed as measles but her correct final diagnosis was Kawasaki disease (KD); she very rapidly defervesced and regained normal function of her limbs after appropriate treatment. We believe this is the first reported case in Liberia of KD in a child younger than two years of age.

Atypical desquamation in a 2.5-year-old boy with Kawasaki disease: A case report.

Kawasaki disease (KD) is a vasculitis that mostly affects children under 5 years of age. This article presents a 2.5-year-old boy who presented with 6 days of fever, generalized maculopapular rash, bilateral non-exudative conjunctivitis, cracked lips, right cervical lymphadenopathy, erythematous extremities, and perianal desquamation. Laboratory studies showed leukocytosis and sterile pyuria. Because diagnosis of KD was proved, oral acetylsalicylic acid with the anti-inflammatory dose and intravenous immunoglobulin were started for him. On the seventh day of admission time, he developed desquamation and erythema on the site of his right cervical lymphadenopathy as well as periungual scaling. About three weeks after starting the treatment, scaling of the cervical lymphadenopathy and periungual area stopped. Echocardiography was performed for him three times: at the time of diagnosis, four weeks, and 6 months later and revealed normal coronary arteries. We report this sign, desquamation on the site of cervical lymphadenopathy, as a new finding.

First_Aid_Step2

Rubella Rubella virus Prodrome: Asymptomatic or tender, generalized lymphadenopathy. Rash: Presents with an erythematous, tender maculopapular rash that also starts on the face and spreads distally. In contrast to measles, children with rubella often have only a low-grade fever and do not appear as ill. Polyarthritis may be seen in adolescents. Encephalitis, thrombocytopenia (a rare complication of postnatal infection). Congenital infection is associated with congenital anomalies. Roseola infantum HHV-6 Prodrome: Acute onset of high fever (> 40°C); no other symptoms for 3–4 days. Rash: A maculopapular rash appears as fever breaks (begins on the trunk and quickly spreads to the face and extremities) and often lasts < 24 hours. Febrile seizures may occur as a result of rapid fever onset. Varicella Varicella-zoster virus (VZV) Prodrome: Mild fever, anorexia, and malaise precede the rash by 24 hours. Rash: Generalized, pruritic, “teardrop” vesicular periphery; lesions are often at

Scarlet Fever -- History and Physical

Typically, scarlet fever is associated with acute pharyngitis. As a result, fever, sore throat, pain with swallowing, and cervical adenopathy is present. If there is no pharyngitis, the source of infection can be a wound or burn which is infected with GAS. The two vectors of infection can both cause scarlet fever and are not distinguishable from one another. The rash itself is a blanching, papular rash. It is distinguished from the macular rash found an allergic reaction by its insidious emergence and lack of confluence of the lesions. This lack of confluence is the primary reason it feels like sandpaper. Also of note, there are no vesicles or pustules present. Vesicles are more associated with the “dew on a rose petal” appearance of chickenpox in its initial stages. Pustules are more indicative of a local infection such as impetigo or erysipelas. The rash develops within 2 to 3 days after infection but can be delayed up to 7 days. The trunk, underarms, and groin are affected first, and then it spreads to the extremities. Usually, the palms and soles are spared. The circumoral area is also spared, making it pallor-like. The “strawberry tongue” begins with a white coating of the tongue with hyperplastic papillae. As the white coating resolves, the papules remain, giving the appearance of a strawberry. Pastia lines are found in the folds of the skin such as the neck, antecubital fossa, and groin. This appears as a linear accumulation of papules around pressure points. After the initial rash begins to resolve, a period of desquamation can occur and last up to two weeks in some cases.

Kawasaki disease

The course of the disease can be divided into three clinical phases. The acute febrile phase, which usually lasts for one to two weeks, is characterized by fever, conjunctival injection, erythema of the oral mucosa, erythema and swelling of the hands and feet, rash, cervical adenopathy, aseptic meningitis, diarrhea, and hepatic dysfunction. Myocarditis is common during this time, and a pericardial effusion may be present. Coronary arteritis may be present, but aneurysms are generally not yet visible by echocardiography. The subacute phase begins when fever, rash, and lymphadenopathy resolve at about one to two weeks after the onset of fever, but irritability, anorexia, and conjunctival injection persist. Desquamation of the fingers and toes and thrombocytosis are seen during this stage, which generally lasts until about four weeks after the onset of fever. Coronary artery aneurysms usually develop during this time, and the risk for sudden death is highest.

Rubeola (Measles) -- History and Physical

The clinical picture of measles can be divided into three stages:  prodromal, eruptive, and convalescent and should be suspected in patients with the classic triad of the three “Cs”:  cough, conjunctivitis, and coryza. The primary or prodromal phase lasts four to six days and is characterized by the presence of high fever, malaise, coryza, conjunctivitis, palpebral edema, and dry cough.  Most cases show the characteristic Koplik spots of the disease, located in the buccal mucosa at the height of the second molar, and appear two to three days before the rash and disappear on the third day. The second phase, the eruptive, is characterized by the appearance of a maculopapular rash, initially fine that subsequently becomes confluent. The rash begins behind the auricle and along the hair implantation line, and extends downward to the face, trunk, and extremities. The third phase or convalescence occurs after three to four days when the rash begins to disappear, in the same order in which it appeared, leaving brown spots and producing a thin peeling of the skin. The fever disappears two to three days after the rash begins, as does the general malaise.

Scarlet fever

Drug eruption: These are potential side effects of taking certain drugs such as penicillin. The reddened maculopapular rash which results can be itchy and be accompanied by a fever. Kawasaki disease: Children with this disease also present a strawberry tongue and undergo a desquamative process on their palms and soles. However, these children tend to be younger than 5 years old, their fever lasts longer (at least five days), and they have additional clinical criteria (including signs such as conjunctival redness and cracked lips), which can help distinguish this from scarlet fever. Toxic shock syndrome: Both streptococcal and staphylococcal bacteria can cause this syndrome. Clinical manifestations include diffuse rash and desquamation of the palms and soles. It can be distinguished from scarlet fever by low blood pressure, lack of sandpaper texture for the rash, and multi-organ system involvement.


In this patient, he presents with a granulomatosis with polyangiitis formerly known as Wegener's granulomatosis. In the clinical case we are told about the otorhinolaryngologic involvement that is present in 92% of patients. At the pulmonary level, we were told that mucus with a clot was present in 85% of the patients. Also at the end of the case, they comment us the urinalysis with a glomerulonephritis that is present in 77% of the patients.

A 67-year-old man presents with 3 months of asthenia and febrile fever, with nasal obstruction and mucus emission with some clots in the last month. In the last few days she noticed pain in the right eye and asymmetry with respect to the contralateral eye. Physical examination reveals proptosis of the right eyeball and inspection of the nostrils reveals an erythematous mucosa with serohematic crusts. The rest of the examination was normal. Blood tests (hemogram, renal and hepatic function) are normal, except for an ESR of 65 mm/h; urinalysis shows microhematuria and proteinuria of 520 mg/24h. What is the most probable initial diagnosis?

Eosinophilic granulomatosis with polyangiitis.

Systemic vasculitis

Medium vessel vasculitis These conditions are sometimes considered together with the small vessel vasculitides. Polyarteritis nodosa (PAN). Systemic necrotizing vasculitis and aneurysm formation affecting both medium and small arteries. If only small vessels are affected, it is called microscopic polyangiitis, although it is more associated with granulomatosis with polyangiitis than to classic PAN. At least 3 out of 10 criteria yields sensitivity and specificity of 82 and 87%: Unexplained weight loss > 4 kg Livedo reticularis Testicular pain Myalgias, weakness Abdominal pain, diarrhea, and GI bleeding Mononeuropathy or polyneuropathy New onset diastolic blood pressure > 90 mmHg Elevated serum blood urea nitrogen (> 40 mg/dL) or serum creatinine (> 1.5 mg/dL) Hepatitis B infection Arteriographic abnormalities Arterial biopsy showing polymorphonuclear cells

Eosinophilic granulomatosis with polyangiitis as a rare cause of the syndrome of inappropriate antidiuretic hormone secretion.

Eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) is a rare multisystem necrotizing vasculitis that involves small- to medium-sized blood vessels. We report a rare case of syndrome of the inappropriate antidiuretic hormone (ADH) secretion (SIADH) secondary to EGPA. A 53-year-old man applied with complaints of pain in the large joints and morning stiffness in knee for 2 months. The patient had the history of impaired fasting glucose, asthma, nasal polyps, and urticaria. Physical examination revealed intrinsic muscle atrophy and weakness in the right hand. Peripheral eosinophil count was 9.78 × 109/L (0.02-0.5), erythrocyte sedimentation rate 39 mm/h (0-20), and C-reactive protein 5.77 mg/dL (0-0.5). Migratory ground-glass pulmonary opacities had been reported in previous chest computed tomography scans. Echocardiography revealed findings compatible with eosinophilic involvement. Electroneuromyographic evaluation showed acute distal axonal neuropathy of right ulnar nerve. EGPA was considered. Oral methylprednisolone treatment was initiated. Intravenous immunoglobulin (IVIG) and cyclophosphamide treatment and gradual tapering of oral steroids were planned. In 24-h urine analysis, sodium was 387 mEq, creatinine was 1156 mg, and volume was 3000 mL. When his medical records were investigated, it was observed that hyponatremia was present for nearly 2 years. While serum osmolality was 270, urine osmolality was 604 mOsm/kg H<sub2</subO. So, SIADH diagnosis was made. Fluid intake was restricted. Although the patient's sodium level did not return to normal, it rose up to 130 mEq/L. After second cycle of EGPA treatment (cyclophosphamide and IVIG), serum sodium was normal. There is only four other documented cases of SIADH associated with EGPA. We hypothesized that blood supply to the hypothalamus and/or posterior hypophysis might be affected from EGPA vasculitis. Here, in this case, with effective treatment of EGPA, SIADH was resolved which implies a causality between two conditions.

Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).

Churg-Strauss syndrome, Eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis that affects small- to medium-sized vessels. It is rare and part of the Anti-neutrophil cytoplasm antibody-associated vasculitis (ANCA) group. We present a 37-year-old man, with a previous history of asthma, that was sent to the ED due to 2 weeks of productive cough, occasional dyspnea on exertion, fever (one week), asthenia, and anorexia. Upon physical examination, he was subfebrile and tachycardic. He had leukocytosis (17.00 x10^9/L) and eosinophilia of 20.0 % (3.4 X10^9/L), creatinine level of 1.5 mg/dL, subtle elevation on liver function tests and CRP of 10.82mg/dL. On Chest X-Ray, there was infiltrate on the right pulmonary base. Due to a strong suspicion of EGPA, he was started on 80mg of prednisolone from admission. ANCA MPO was positive, with the remaining auto-immune study negative. He underwent Thorax CT (under corticotherapy) without relevant changes, as well as bronchoalveolar lavage, without macroscopic signs of alveolar hemorrhage. Because of active urinary sediment, nephrotic proteinuria (6.5g/24h), and acute renal failure he underwent a renal biopsy, which revealed pauci-immune crescentic glomerulonephritis, with predominantly acute findings (in the context of ANCA-MPO Vasculitis - EGPA). After the biopsy, he received three 1g methylprednisolone pulses and was started on Cyclophosphamide. He remained asymptomatic and renal function was restored. This case highlights the importance of integrating all findings in one clinical scenario to prevent a more complex disease diagnosis, with a specific treatment, from being missed.

Granulomatosis with polyangiitis: seeing the diagnosis.

A 41-year-old woman presented to her primary doctor with nausea, back pain and lower extremity oedema. Initial labs showed elevated serum creatinine and white blood cell count (WBC), which her doctor attributed to ibuprofen use and a recent upper respiratory infection. Five days later, she presented to the eye clinic with eye pain, redness and blurred vision. She was diagnosed with iritis, conjunctivitis and keratitis. The inflammatory eye disease with decreased renal function prompted the ophthalmologist to initiate systemic autoimmune and infectious disease work-up. Before laboratory testing was complete, she developed severe haemoptysis. Diagnosis of granulomatosis with polyangiitis (GPA) was confirmed using blood testing, radiological imaging and kidney biopsy. She received plasmapheresis, then cyclophosphamide and prednisone with good effect. This case highlights the need to consider GPA in the differential when patients present with inflammatory eye disease with decreased renal function and the need for multispecialty collaboration including ophthalmologists in the diagnosis of GPA.

Systemic vasculitis

Lab tests. Basic lab tests may include a complete blood count, chemiestries (look for creatinine), creatine phosphokinase level, liver function tests, erythrocyte sedimentation rate, hepatitis serologies, urinalysis, chest X-ray, and an electrocardiogram. Additional, more specific tests include: Antinuclear antibody test can detect an underlying connective tissue disorder, especially lupus erythematosus Complement levels that are low can suggest mixed cryoglobulinemia, hepatitis C infection, and lupus erythematosus], but not most other vasculitides. Antineutrophil cytoplasmic antibody may suggest granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, or drug-induced vasculitis, but is not diagnostic. Electromyography. It is useful if a systemic vasculitis is suspected and neuromuscular symptoms are present.

Granulomatosis with polyangiitis-associated ischemic optic neuropathy in a previously healthy 50-year-old female.

To describe a case of anterior ischemic optic neuropathy as a presenting sign of granulomatosis with polyangiitis. A previously healthy 50-year-old female developed right eye, then left eye, redness and pruritis and was diagnosed with allergic versus viral conjunctivitis. Five days later, she noted an acute decline in vision in the right eye, corresponding with a decrease on Snellen testing from 20/30 to 20/100 with correction. She was noted to have a right relative afferent pupillary defect, 2+ pallid disc edema, and OCT (Spectralis, Heidelberg Engineering, Carlsbad, CA) findings of significant retinal nerve fiber thickening. Review of systems revealed a three-month history fatigue, right-sided headaches, jaw claudication, bronchitis, cough without hemoptysis, and epistaxis, as well as interval development of a petechial rash across her body, migratory polyarthralgias, fevers, and tachycardia. ESR and CRP were markedly elevated, and the patient was admitted to the hospital for a systemic vasculitis workup. She was started on IV methylprednisolone. Her vision improved dramatically with steroids, measuring 20/50 with correction in the right eye after 24 hours and returning to baseline after five days. An extensive workup including imaging, bloodwork, and biopsies led to a diagnosis of granulomatosis with polyangiitis, with PR3-positive ANCA. Ocular findings, including anterior ischemic optic neuropathy, may be the presenting signs for patients with granulomatosis with polyangiitis. Prompt recognition and treatment with high-dose steroids and immunomodulatory therapy is important for visual recovery. Prompt recognition of potential vasculitis-related vision loss can lead to timely initiation of vision-saving treatment.

Vasculitis-like hemorrhagic retinal angiopathy in Wegener's granulomatosis.

Granulomatosis with polyangiitis, also known as Wegener's granulomatosis, is a chronic systemic inflammatory disease that can also involve the eyes. We report a case of massive retinal and preretinal hemorrhages with perivascular changes as the initial signs in granulomatosis with polyangiitis (Wegener's granulomatosis). A 39-year-old Caucasian male presented with blurred vision in his right eye, myalgia and arthralgia, recurrent nose bleeds and anosmia. Fundus image of his right eye showed massive retinal hemorrhages and vasculitis-like angiopathy, although no fluorescein extravasation was present in fluorescein angiography. Laboratory investigations revealed an inflammation with increased C-reactive protein, elevated erythrocyte sedimentation rate and neutrophil count. Tests for antineutrophil cytoplasmic antibodies (ANCA) were positive for c-ANCA (cytoplasmatic ANCA) and PR3-ANCA (proteinase 3-ANCA). Renal biopsy demonstrated a focal segmental necrotizing glomerulonephritis. Granulomatosis with polyangiitis (Wegener's granulomatosis) was diagnosed and a combined systemic therapy of cyclophosphamide and corticosteroids was initiated. During 3 months of follow-up, complete resorption of retinal hemorrhages was seen and general complaints as well as visual acuity improved during therapy. Vasculitis-like retinal changes can occur in Wegener's granulomatosis. Despite massive retinal and preretinal hemorrhages that cause visual impairment, immunosuppressive therapy can improve ocular symptoms.

Polyarteritis nodosa

Diagnosis No specific lab tests exist for diagnosing polyarteritis nodosa. Diagnosis is generally based on the physical examination and a few laboratory studies that help confirm the diagnosis: CBC (may demonstrate an elevated white blood count) ESR (elevated) Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not associated with "classic" polyarteritis nodosa, but is present in a form of the disease affecting smaller blood vessels, known as microscopic polyangiitis or leukocytoclastic angiitis Tissue biopsy (reveals inflammation in small arteries, called arteritis) Elevated C-reactive protein

Microscopic polyangiitis

Diagnosis Laboratory tests may reveal an increased sedimentation rate, elevated CRP, anemia and elevated creatinine due to kidney impairment. An important diagnostic test is the presence of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) with myeloperoxidase specificity (a constituent of neutrophil granules), and protein and red blood cells in the urine. In patients with neuropathy, electromyography may reveal a sensorimotor peripheral neuropathy. Differential diagnosis The signs and symptoms of microscopic polyangiitis may resemble those of granulomatosis with polyangiitis (GPA) (another form of small-vessel vasculitis) but typically lacks the significant upper respiratory tract involvement (e.g., sinusitis) frequently seen in people affected by GPA.

[Disseminated histoplamosis in adolescent mimicking granulomatosis with polyangiitis].

Systemic histoplasmosis is an invasive fungal infection that may mimic primary vasculitis, particularly granulomatosis with polyangiitis (GPA), and was rarely described in adult patients. We reported an immunocompetent patient with disseminated histoplasmosis mimicking GPA who fulfilled European League Against Rheumatism (EULAR)/Pediatric Rheumatology International Trials Organisation (PRINTO)/Pediatric Rheumatology European Society (PRES) validated classification criteria. A 6-year old boy presented acute migratory polyarthritis with spontaneous improvement, sinus inflammation, fever, headache and abdominal pain. Serologic test for hepatitis, cytomegalovirus, human immunodeficiency virus, Epstein-Barr virus, toxoplasmosis, dengue virus and antistreptolysin O were all negative. Magnetic resonance imaging (MRI) showed moderate ascites in pelvis and pansinusitis. Antineutrophil cytoplasmic antibodies (c-ANCA) were positive. He had spontaneous remission of the symptoms including fever. At the age of 11 years and 11 months, he had sinusitis, pneumonia and epididymitis. A month later, he was hospitalized and MRI showed left eye proptosis. Cerebrospinal fluid was normal and indirect tests of fungi were negative. Two months later, he had lumbar pain and computer tomography showed a mass in the right kidney and pulmonary nodule in the right lung. He fulfilled EULAR/PRINTO/PRES criteria for GPA, however the renal biopsy showed a focal granulomatous interstitial nephritis with yeast fungal cells compatible with Histoplasma sp. He was treated with liposomal amphotericin B and itraconazole with improvement of signs and symptoms. We reported a progressive disseminated histoplasmosis case mimicking GPA. Histoplasmosis infection should be considered in immunocompetent subjects with uncommon clinical manifestations, such as arthritis, nephritis and epididymitis.

The role of conjunctival biopsy in the diagnosis of granulomatosis with polyangiitis.

The purpose of this study is to describe a patient who was diagnosed with granulomatosis with polyangiitis based on conjunctival biopsy. This study is a case report and review of the literature. A 48-year-old Caucasian woman presented with a 2-week history of a left eye peripheral corneal ulcer with adjacent conjunctivitis and a 4-month history of a non-resolving productive cough. Given her elevated serum perinuclear antineutrophil cytoplasmic antibody (P-ANCA) and erythrocyte sedimentation rate (ESR) levels as well as a chest computed topography (CT) that showed bilateral patchy infiltrates, suspicion of limited granulomatosis with polyangiitis with lung and ocular involvement was high. Because bronchoalveolar lavage was nondiagnostic for granulomatous disease, conjunctival biopsy was initially attempted in order to avoid a more invasive lung biopsy. The conjunctival biopsy revealed mixed subacute inflammatory mediators and vasculitis consistent with granulomatosis with polyangiitis. Conjunctival biopsy may be a valuable, minimally invasive method for diagnosing systemic granulomatosis with polyangiitis.

[Spontaneous kidney rupture as an early complication of Wegener's granulomatosis].

A 51-year-old woman had been suffering from blood-stained purulent sinusitis and antibiotic-resistant bouts of fever for 4 months. She had microhematuria and serological evidence of inflammation (erythrocyte sedimentation rate [ESR] 92/135 mm, C-reactive protein 5.0 mg/dl). When she was admitted to hospital suspected of having postinfectious glomerulonephritis she complained of spontaneous colic-like pains in the left flank. Within one day the haemoglobin concentration fell from 10 to 6.5 g/dl. Ultrasound and computed tomography demonstrated a large space-occupying lesion around the left kidney. At operation this was found to be a rupture of the kidney with perirenal bleeding which was treated without removing the kidney. No biopsy was taken, but serological tests showed antineutrophil cytoplasmatic antibodies (cANCA), indicating Wegener's granulomatosis as the cause of the compensated renal insufficiency and spontaneous renal rupture. Under immunosuppressive treatment the inflammatory signs (ESR 18/44 mm), fever, chronic maxillary sinusitis, raised serum creatinine concentration and the ANCA titre all regressed, while proteinuria of about 4 g/24 h persisted. There was no recurrence during a follow-up period of 15 months. Serological signs of marked inflammatory activity, urinary sediments of nephritis and spontaneous retroperitoneal bleeding should suggest that, in addition to lupus erythematodes and panarteritis nodosa, Wegener's granulomatosis be included in the differential diagnosis.

Palpable purpura

Palpable purpura is a condition where purpura, which constitutes visible non-blanching hemorrhages, are raised and able to be touched or felt upon palpation. It indicates some sort of vasculitis secondary to a serious disease. Causes Rocky mountain spotted fever Acute meningococcemia Disseminated gonococcal infection Ecthyma gangrenosum Henoch–Schönlein purpura Eosinophilic granulomatosis with polyangiitis Polyarteritis nodosa Leucocytoclastic vasculitis Microscopic polyangiitis Mixed essential cryoglobulinemia Subacute bacterial endocarditis Diagnosis Identification of underlying cause. Treatment Treat the underlying disease. References Further reading Vascular-related cutaneous conditions

Optic neuropathy secondary to granulomatosis with polyangiitis in a patient with Graves' disease: a case report.

Dysthyroid optic neuropathy is the most commonly suspected diagnosis of optic neuropathy in Graves' patients; however, other causes need to be ruled out. We present a unique case of optic neuropathy secondary to hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be antithyroid drug related. A 79-year-old Japanese male presented with acute visual loss in the left eye. He had a 24-year history of Graves' disease and was taking methimazole. Best-corrected visual acuity was 0.8 in the right eye and light perception in the left eye, and relative afferent pupillary defect in the left eye was seen. Ocular movement was normal, and there were no findings explaining visual loss in intermediate optic media and fundus in the left eye. Contrast-enhanced magnetic resonance imaging demonstrated thickened dura mater. Tests for myeloperoxidase-antineutrophil cytoplasmic antibody, proteinuria, and hematuria were positive; pulmonary nodule lesions and a blood clot in the left lower leg were also found. After excluding the presence of diseases that could lead to hypertrophic pachymeningitis, we diagnosed optic neuropathy due to hypertrophic pachymeningitis with granulomatosis with polyangiitis-a subtype of antineutrophil cytoplasmic antibody-associated vasculitis. Since he had history of using methimazole, antineutrophil cytoplasmic antibody-associated vasculitis was considered as drug related. We started high-dosage steroid pulse therapy followed by 1 mg/kg body weight daily of oral prednisolone, and subsequently tapered. Methimazole was stopped. Best-corrected visual acuity recovered to 0.9, 2 weeks after starting treatment. Though myeloperoxidase-antineutrophil cytoplasmic antibody remained negative, the symptom relapsed 6 months after treatment initiation. We gave a second high-dose steroid pulse therapy followed by prednisolone tapered together with methotrexate. Remission remained, and using 4 mg/week methotrexate without prednisolone, myeloperoxidase-antineutrophil cytoplasmic antibody was kept within the normal limit until now, 4 years after onset. We present a case of optic neuropathy with hypertrophic pachymeningitis related to antineutrophil cytoplasmic antibody-associated vasculitis, which was suspected to be drug related. The patient had good visual recovery after quitting the drug and receiving immunosuppressive therapy with systemic steroids. Hypertrophic pachymeningitis with antineutrophil cytoplasmic antibody-associated vasculitis related to antithyroid drugs should be considered as a differential diagnosis for optic neuropathy in Graves' patients in whom optic nerve compression is not obvious.

Polyarteritis nodosa

Differential diagnosis Polyarteritis nodosa rarely affects the blood vessels of the lungs and this feature can help to differentiate it from other vasculitides that may have similar signs and symptoms (e.g., granulomatosis with polyangiitis or microscopic polyangiitis). Treatment Treatment involves medications to suppress the immune system, including prednisone and cyclophosphamide. When present, underlying hepatitis B virus infection should be immediately treated. In some cases, methotrexate or leflunomide may be helpful. Some patients have entered a remission phase when a four-dose infusion of rituximab is used before the leflunomide treatment is begun. Therapy results in remissions or cures in 90% of cases. Untreated, the disease is fatal in most cases. The most serious associated conditions generally involve the kidneys and gastrointestinal tract. A fatal course usually involves gastrointestinal bleeding, infection, myocardial infarction, and/or kidney failure.

Granulomatosis with polyangiitis

Diagnosis Granulomatosis with polyangiitis is usually suspected only when a person has had unexplained symptoms for a long period of time. Determination of anti-neutrophil cytoplasmic antibodies (ANCAs) can aid in the diagnosis, but positivity is not conclusive and negative ANCAs are not sufficient to reject the diagnosis. More than 90% of people who have GPA test positive for ANCA. Cytoplasmic-staining ANCAs that react with the enzyme proteinase 3 (cANCA) in neutrophils (a type of white blood cell) are associated with GPA. Involvement of the ears, nose, and throat is more common in granulomatosis with polyangiitis than in the similar condition microscopic polyangiitis.

Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review.

Granulomatosis with polyangiitis (GPA), a necrotizing granulomatous disease, very rarely involves the central nervous system (CNS), particularly the pituitary. Delayed treatment may cause permanent bilateral blindness. We report an isolated case of pituitary GPA that manifested as a progressive bilateral temporal visual field (VF) defect and was diagnosed via pituitary biopsy. Additionally, we review ocular, chiasmal and cranial nerve involvement in pituitary GPA. A 20-year-old Chinese man was referred for repeated fever, sudden headache, diplopia with a bilateral best-corrected visual acuity (BCVA) of 10/20, ptosis in both eyes and restricted abduction on the right side. VF tests showed bitemporal hemianopsia. Laboratory tests revealed hypothyroidism and were negative for autoimmune markers. Enhanced magnetic resonance imaging (MRI) showed pituitary enlargement. The diagnosis was lymphocytic pituitaritis. After intravenous (IV) dexamethasone treatment, full recovery occurred within 2 months. Two years later, the patient was readmitted for headache recurrence. With oral prednisone, the visual acuity in his right eye rapidly decreased to hand motion (HM) within one month. Enhanced MRI showed pituitary enlargement and a new, invasive suprasellar CNS lesion. All infection- and autoimmune-related tests were negative. The visual acuity in his right and left eye decreased to no light perception (NLP) after 6 days and 2 weeks, respectively. The biopsy results suggested GPA. After IV methylprednisolone treatment, complete remission of the symptoms occurred and was confirmed by MRI. The 15-month follow-up showed no signs of recurrence. GPA typically affects the respiratory tract, lungs and kidneys. To date, 50 cases with pituitary involvement have been reported. Chiasmal and cranial nerve involvement leading to visual acuity impairment are common. We found 2 cases with severe visual loss resembling our case and discuss certain similarities.

Risk stratification The French Vasculitis Study Group has developed a five-point system ("five-factor score") that predicts the risk of death in Churg–Strauss syndrome using clinical presentations. These factors are: Reduced renal function (creatinine >1.58 mg/dl or 140 μmol/l) Proteinuria (>1 g/24h) Gastrointestinal hemorrhage, infarction, or pancreatitis Involvement of the central nervous system Cardiomyopathy Having none of these factors indicates milder case, with a five-year mortality rate of 11.9%. The presence of one factor indicates severe disease, with a five-year mortality rate of 26%, and three or more indicate very severe disease: 46% five-year mortality rate.

Pauci-immune

In the setting of systemic vasculitis as described above, proliferative nephritis is associated with antineutrophil cytoplasmic antibodies (ANCA). Because of this, an ANCA test should always follow a negative immunofluorescence result to have the highest accuracy for confirming pauci-immune vasculitis-driven proliferative nephritis. Some cases of pauci-immune proliferative nephritis have no explanation and are thus deemed "idiopathic." Peak incidences in 50- to 60-year-olds symptoms include intermittent fever / weight loss / shortness of breath / joint pain. See also Systemic vasculitis#Pauci-immune Goodpasture Syndrome and Poststrep Glomerulonephritis Microscopic polyangiitis, Eosinophilic granulomatosis with polyangiitis or Granulomatosis with polyangiitis References External links wikt:paucity Vascular-related cutaneous conditions

Granulomatosis With Polyangiitis -- History and Physical -- Organ System Involvement

Upon presentation, renal involvement is noted in only 10%-20%, but glomerulonephritis eventually develops in 80% of patients within two years of disease onset. The most common manifestation is rapidly progressive crescentic glomerulonephritis leading to chronic kidney disease or end-stage renal disease. Eye involvement:

[A case of polyarteritis nodosa with bilateral hilar lymphadenopathy].

A 55-year-old male was admitted with non productive cough and fever which had continued for 6 weeks. The patient had symptoms of peripheral neuralgia. Chest X-ray revealed bilateral hilar lymphadenopathy (BHL) and reticular shadows in both lung fields. Other laboratory abnormalities included hematuria, RBC cast, high BUN, leukocytosis and thrombocytosis. Destruction of the internal membrane of arterioles was observed in a livedo reticularis on the right lower extremity. Renal angiography showed irregularity in the diameter, discontinuation and narrowing of peripheral arteries of both kidneys. These findings suggested the existence of "angiitis". These data were compatible with the diagnosis of polyarteritis nodosa (PN). Prednisolone (60 mg/day) administration resulted in the improvement of his symptoms and laboratory findings. A case of PN with lymph node swelling has been reported, however PN with BHL has not yet been reported. This is the first report of PN with BHL.

Classical polyarteritis nodosa and microscopic polyarteritis with medium vessel involvement--a comparison of the clinical and laboratory features.

Microscopic polyarteritis may involve medium-sized and small blood vessels as well as arterioles, venules and capillaries. We have compared the clinical and laboratory features in patients with microscopic polyarteritis and medium vessel involvement, with the features found in patients with polyarteritis nodosa affecting medium vessels alone. In a 9-year period, 21 patients presented to our hospital with a form of polyarteritis. Seven had microscopic polyarteritis demonstrated histologically (6/7, 86%) and associated with dysmorphic urinary red cells (7/7, 100%), as well as medium vessel vasculitis demonstrated histologically (7/7) or by angiography (1/7, 14%). Five patients had polyarteritis nodosa with medium vessel vasculitis demonstrated histologically (3/5, 60%) or by angiography (2/5, 40%); and no evidence of a glomerular vasculitis on biopsy (2/7, 29%) or in the urinary sediment (0/7, 0%). The remaining 9 patients had microscopic polyarteritis but medium vessel involvement was not excluded by angiography. All patients with microscopic polyarteritis and medium vessel involvement had glomerular hematuria (> 100,000 glomerular RBC/ml), proteinuria > 0.5 g/24 hours), and an elevated serum creatinine (0.166 to 0.811 mmol/l). Other symptoms included fever (6/7, 86%), night sweats (5/7, 71%), gastrointestinal bleeding (4/7, 57%), proximal myopathy (3/7, 43%) and peripheral neuropathy (3/7, 43%). One patient (1/7, 14%) had hypertension. Anemia (6/7, 86%), a raised ESR (6/7, 86%), thrombocytosis (6/7, 86%), hypoalbuminemia (6/7, 86%) and abnormal liver function tests (6/7, 86%) were common. Two patients (29%) had an eosinophilia. All 5 individuals who were tested for ANCA were positive (2cANCA, 2pANCA and one pattern not described). In contrast, in patients with polyarteritis nodosa and medium vessel involvement alone, an elevated ESR was common (4/5, 80%) but fever (1/5, 20%), night sweats (0/5, 0%), proximal myopathy (1/5, 20%) and peripheral neuropathy (1/5, 20%) were seen infrequently; hypertension (1/5, 20%) and eosinophilia (1/5, 20%) were also uncommon; and ANCA were not demonstrated (0/3, 0%). Medium-sized vessel involvement is common in patients with microscopic polyarteristis, and these patients are more likely to have renal involvement and systemic symptoms, and be ANCA-positive, than patients with polyarteritis nodosa alone. Gastrointestinal symptoms are often seen in both groups.

Systemic vasculitis

Mononeuritis multiplex. Also known as asymmetric polyneuropathy, in a non-diabetic this is suggestive of vasculitis. Palpable purpura. If patients have this in isolation, it is most likely due to cutaneous leukocytoclastic vasculitis. If the purpura is in combination with systemic organ involvement, it is most likely to be Henoch–Schönlein purpura or microscopic polyangiitis. Pulmonary-renal syndrome. Individuals who are coughing up blood and have kidney involvement are likely to have granulomatosis with polyangiitis, microscopic polyangiitis, or anti-GBM disease (Goodpasture syndrome). Diagnosis A detailed history is important to elicit any recent medications, any risk of hepatitis infection, or any recent diagnosis with a connective tissue disorder such as systemic lupus erythematosus (SLE). A thorough physical exam is needed as usual.

[Microscopic polyangiitis with eosinophilia--an overlap syndrome or separate disease entity? A case report and review of the literature].

Systemic vasculitides are potentially life-threatening diseases. Early and appropriate diagnosis based on case history, clinico-pathological features, and laboratory parameters, such as the presence of anti-neutrophil cytoplasmic antibodies (ANCA), is crucial for starting appropriate and, often, life-saving therapeutic measures. We report a 50-year-old female patient who presented with fever, arthralgias and hemoptysis. Skin signs included disseminated hemorrhagic pustules, ulcerations of oral and genital mucosa, subcutaneous nodules on arms and legs, and a pyoderma gangrenosum-like lesion on the right leg. Laboratory investigations revealed a peripheral eosinophilia and a positive cANCA titer. Histopathologic analysis of various biopsy specimens showed a granulomatous vasculitis in the subcutis, a nongranulomatous vasculitis with massive eosinophil infiltration in the lungs, and a segmental, necrotizing glomerulonephritis in the kidneys. Differential diagnosis included Wegener's granulomatosis, microscopic polyangiitis (MPA) and Churg-Strauss syndrome. MPA was diagnosed based on clinical and histopathological criteria. An interesting feature of this case was marked peripheral and tissue eosinophilia. Therapy consisted of cyclophosphamide and methylprednisolone. The patient went into a long-lasting clinical remission one month after starting therapy.

Wegener's granulomatosis with orbital involvement: case report and literature review.

<bObjective:</b To describe the chronology and the extent of orbital involvement in a case of granulomatosis with polyangiitis. <bMethods:</b Descriptive case report and literature review. <bResults:</b A 45-year-old patient, formerly diagnosed with granulomatosis with polyangiitis due to otorhinolaryngologic manifestations, pulmonary lesions, renal impairment, left knee arthritis and high blood levels of antineutrophil cytoplasmic antibodies, addressed the Ophthalmology Department in November 2020, having the following complaints: left eye mild retro-orbital discomfort, proptosis and epiphora. On examination, Snellen's best corrected visual acuity was 6/ 6 in both eyes. The anterior segment of the left eye displayed significant changes: proptosis, upper lid swelling, ptosis, slightly decreased ocular motility, mild conjunctival hyperemia and chemosis, thinning of sclera in the upper quadrant and mild posterior subcapsular cataract. Left eye funduscopy revealed a slightly elevated optic disc, with indistinct margins in the nasal quadrant. Spectral-domain optical coherence tomography (OCT) of the optic nerve confirmed the clinical findings, illustrating an increase in the retinal nerve fiber layers thickness in the nasal quadrant, with no corresponding visual field defect. The orbit magnetic resonance imaging (MRI) unveiled an intraconal mass surrounding the optic nerve on its entire intra-orbital path, confirming the diagnosis of left orbital granuloma. <bConclusion:</b Considering the relapsing disease and the orbital involvement, the patient is currently a candidate for rituximab, a monoclonal antibody against CD20. <bAbbreviations:</b AAV = ANCA associated vasculitides, ANCA = antineutrophil cytoplasmic antibody, AOM = acute otitis media, BCVA = best corrected visual acuity, CRP = C-reactive protein, CT = computerized tomography, EGPA = eosinophilic granulomatosis with polyangiitis, ENT = otorhinolaryngology/ ear-nose-throat, ESR = erythrocyte sedimentation rate, GPA = granulomatosis with polyangiitis, LE = left eye, MPA = microscopic polyangiitis, MRI = magnetic resonance imaging, OCT = optical coherence tomography, RE = right eye, RNFL = retinal nerve fiber layers, TNF = tumor necrosis factor, WG = Wegener's granulomatosis.

[Intracranial multiple granuloma preceded by rheumatic disease--a case report].

In 1980, a 38-year-old man had remittent fever, swelling and arthralgia of the knee, ankle and wrist joints, as well as visual disturbance due to bilateral iritis. On his admission to our hospital, his laboratory data showed neutrophilia, normocytic normochromic anemia, hepatomegaly, hepatocellular damage, and a strongly positive RA test. All the microbiological examinations were negative. Thirty mg of prednisolone improved his symptoms and abnormal laboratory findings. Due to persistent mild arthralgia, he had continued to take 5-10 mg of prednisolone and analgesics until 1985 when he was readmitted to our hospital. In 1983, he began to complain of a steady pain around his left eye, and he sometimes had double vision. In 1985, he began to complain of decreasing left visual acuity and sensory disturbance in his left face in addition to pain in and around the eye. On his 2nd admission to our hospital, the neurological examination revealed involvement of the 2nd and 3rd cranial nerves and the 1st branch of the 5th cranial nerves of the left side. Laboratory data showed a positive RA test with RAHA titer at 1:320 and IgM at 216 mg/dl, but he had no joint deformities. The computed tomography (CT) of the brain demonstrated a high density mass of his left cavernous sinus extending to the left orbital apex. The prescription of the high dose of prednisolone (100 mg/day) relieved ophthalmic pain and improved visual acuity and neurological involvement within a week. Prednisolone was then gradually decreased to 10 mg. In 1986, he had partial and complex partial seizures.(ABSTRACT TRUNCATED AT 250 WORDS)

Rheumatology

Systemic conditions and connective tissue diseases Lupus Ehlers-Danlos syndrome Sjögren's syndrome Scleroderma (systemic sclerosis) Polymyositis Dermatomyositis Polymyalgia rheumatica Mixed connective tissue disease Relapsing polychondritis Adult-onset Still's disease Sarcoidosis Fibromyalgia Myofascial pain syndrome Vasculitis Microscopic polyangiitis Eosinophilic granulomatosis with polyangiitis Granulomatosis with polyangiitis Polyarteritis nodosa Henoch–Schönlein purpura Serum sickness Giant cell arteritis, Temporal arteritis Takayasu's arteritis Behçet's disease Kawasaki disease (mucocutaneous lymph node syndrome) Thromboangiitis obliterans Hereditary periodic fever syndromes

Conjunctivitis as the important indicator of pediatric granulomatosis with polyangiitis.

Granulomatosis with polyangiitis disease is a rare vasculitis characterized by granulomatous inflammation of respiratory tracts and glomerulonephritis along with vasculitis of other organs. In this study, a 14- year-old boy was referred from ophthalmology clinic to the pediatric rheumatology ward due to drug-resistant conjunctivitis. He had a history of chronic rhinorrhea and nighttime coughing, and he was diagnosed with allergic rhinitis. Complete blood count showed leukocytosis and thrombocytosis, and the estimated sedimentation rate was elevated. Laboratory tests showed hematuria, proteinuria, and highly positive antineutrophil cytoplasmic antibody. Moreover, sinus computed tomography demonstrated pansinusitis, and spiral chest computed tomography showed multiple pulmonary nodules in both his lungs. Finally, based on renal biopsy, the patient was confirmed as a case of granulomatosis with polyangiitis. It is notable that acute or chronic conjunctivitis may be a manifestation of rheumatic diseases.

Vasculitis

A small number have been shown to have a genetic basis. These include adenosine deaminase 2 deficiency and haploinsufficiency of A20. According to the size of the vessel affected, vasculitis can be classified into: Large vessel: Takayasu's arteritis, Temporal arteritis Medium vessel: Buerger's disease, Kawasaki disease, Polyarteritis nodosa Small vessel: Behçet's syndrome, Eosinophilic granulomatosis with polyangiitis, Cutaneous vasculitis, granulomatosis with polyangiitis, Henoch–Schönlein purpura, and microscopic polyangiitis. Condition of some disorders have vasculitis as their main feature. The major types are given in the table below: Takayasu's arteritis, polyarteritis nodosa and giant cell arteritis mainly involve arteries and are thus sometimes classed specifically under arteritis.

Laboratory testing in the evaluation and diagnosis of vasculitis.

A multitude of tests are available for the diagnosis and management of the vasculitides. Most of them are nonspecific but provide useful information that, when appropriately used in conjunction with the patient's history and physical examination can be of great assistance in arriving at a final diagnosis. In addition, information gathered may be of great help in monitoring disease activity and clinical response to therapy, in indicating the presence of specific organ system involvement, in monitoring toxicity of medication used, and in assessing prognosis. Serial measurements of acute phase reactants, complete blood cell count with differential, biochemistry profiles, urinalysis, and C3 and C4 levels should be obtained in all patients. Antineutrophil cytoplasmic antibodies (ANCA) determination provides valuable information and is highly specific for the diagnosis of small-vessel vasculitides, particularly Wegener's granulomatosis and microscopic polyangiitis. ANCA levels can be particularly useful to assess disease activity in these disorders. Hepatitis-B and, more importantly, hepatitis-C testing is extremely useful, particularly in the presence of liver involvement and associated risk factors. Angiographic studies may confirm the diagnosis, particularly if there is laboratory and clinical evidence of specific organ involvement. It should be noted, however, that angiography may be normal even when vasculitis is present, or the findings may be nonspecific. A definite diagnosis is provided by a tissue biopsy. This should be performed whenever there is access to clinically affected tissue.

Eosinophilic granulomatosis with polyangiitis complicated with rapidly progressive glomerulonephritis in a young man who is a healthy cyclist.

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis. Rapidly progressive glomerulonephritis (RPGN) is a rare complication of EGPA. We report a case of a 60-year-old man, who is also a skilled cyclist, who was hospitalized to investigate a symptomatology that had arisen over the previous months and worsened in the last few weeks, to the point of limiting normal everyday activities. The physical examination revealed the presence of livedo reticularis of the four limbs, purpura of the lower limbs, arthritis of the ankles, and low-grade fever; the patient showed intense asthenia, loss of appetite, retrosternal heartburn, and a scarcely pharmacologically controlled asthma. He also reported weight loss (about 5 kg in the last 6 months). Rapidly progressing renal failure was observed with hyper-eosinophilia (4.7 thousand/μL eosinophils, 44% of total leukocytes), pulmonary opacities on chest computed tomography (CT), and sinusitis on CT of the facial massif. The search for antibodies directed against neutrophil cytoplasm (ANCA) revealed a high level of pANCA (pANCA ++, ELISA anti-MPO 666 UI/ml), associated with an increment of inflammation indicators. The induction therapy was high-dosage intravenous glucorticoids and cyclophosphamide, to improve the short and long-term prognosis. After 7 months of treatment, the patient reported a considerable improvement of the symptoms, which at that point did not necessitate pharmacological interventions. The eosinophils value was 0 cells/mm³, the inflammation indexes were back to the norm, and the renal function appeared significantly improved.

[Microscopic polyarteritis].

The microscopic polyarteritis is a vasculitis related to necrotizing glomerolunephritis. It always damages at renal and systemic level (a third of the cases presented hemorrhage alveolar). We have showed a case that took place with hemoptysis and renal progressive insufficiency. Among the patient antecedents, we can find arterial hypertension hematuria, rhinitis, epistaxis and artromyalgias. Just before his admittance it showed edemas on lower limbs and eyelids, dysnea, severe hemoptysis, paresthesias and general malaise. The immunologic analysis: Acs. glomerular basal antimembrane: negative, ANCA positive with antimieloperoxidasa specificity. The renal biopsy: focal necrotizing glomerulonephritis with semilunar and negative immunofluorescent. The nasas biopsy: unspecified chronic rhinitis. From the clinic point of view, the patient seemed to have the Wegener granulomatosis apart from the fact that he had hemoptysis which is a rare sign in this cases. However, we could not find any granuloma anatomopatologically, which did not clarify this diagnosis. We diagnosed microscopis Poliarteritis, as a third of the cases presented intrapulmonary haemorrhage. Moreover the renal damage it is identical than in the in Wegener granulomatosis. On the top of that, often we can find p-ANCA, which confirms the diagnosis in 99% of cases. Despite our doubt according to the diagnosis the therapeutical treatment of both illnesses is nowadays identical. This means that we were able to begin a precocious treatment with plasmapheresis, metilprednisolona and ciclofosfamida. After a week treatment there was an evident improvement. Five moth later the illness relapsed.