intestinal pseudo - obstruction ( ipso ) is defined as ineffective intestinal propulsion which occurs in the absence of mechanical or obstructive factors ( 1 ) .
cases , in whom ipso is the primary clinical feature , are typically associated with connective tissue diseases .
however , ipso presenting as the first manifestation in systemic lupus erythematosus ( sle ) is rare , and < 30 cases of ipso in patients with sle have been reported ( 14 ) .
clinical features generally include severe abdominal symptoms , such as distension , pain , nausea and vomiting , while radiographic signs show gaseous small bowel distension with air - fluid levels .
acute lupus pneumonitis ( alp ) is an uncommon pulmonary manifestation of sle , and lung involvement has only been reported in 3% of sle patients ( 5,6 ) .
alp is an unusual but life threatening complication of sle , and usually presents with the acute onset of fever , cough , tachypnea and hypoxia .
the radiological sign of alp is consolidations in one or more areas , and is often associated with pleural effusion and pulmonary arterial hypertension .
the majority of alp responds to steroids and some requires treatment with cyclophosphamide ; however , the rate of mortality remains high ( 5,7 ) .
ipso and alp occurring in a single patient , described as co - manifestation with sle , has not been reported previously .
herein , we report a case in which ipso was the initial presentation of sle , and secondary onset of alp occurred after emergency surgery .
a 26-year - old female , complaining of abdominal pain , nausea , vomiting and constipation for 1 day , was admitted to the emergency room of the second affiliated hospital of zhejiang university ( hangzhou , china ) in june 2013 .
physical examinations showed distress countenance , soft abdomen , absence of bowel sounds , abdominal tenderness but no rebound tenderness .
1a ) , white blood cell ( wbc ) count , hemoglobin ( hgb ) , erythrocyte sedimentation rate ( esr ) , c - reactive protein ( crp ) and urinalysis were normal .
computed tomography ( ct ) scanning showed that the jejunal wall was thickened and streaky ( fig .
the patient was thought to have strangulated intestinal obstruction , and an emergency laparotomy was performed . during the operation ,
~80 cm of the jejunum showed hyperemia and edema , with multiple petechiae on the serosal surface , and lymph node enlargement in the mesentery .
in addition , ~800 ml turbid peritoneal fluid was detected , but no bowel perforation .
peritoneal lavage , segmental resection of the diseased jejunum and placement of a jejunostomy fistula were performed .
pathological examination of the jejunum using hematoxylin ( shanghai bioscience & technology co. , ltd . , shanghai , china ) and eosin ( shanghai sss reagent co. , ltd . , shanghai , china ) ( h&e ) staining showed chronic inflammation of intestinal mucosa , with submucosal anapetia , congestion , hemorrhage and interstitial edema .
post - operatively , intestinal peristalsis did not recover , and a gastrointestinal decompression tube was placed in the duodenum .
apart from the bowel problem , sporadic rashes and mild fever without clear origin appeared .
following anti - infective treatment ( ceftazidimine , 2.5 g intravenous injection , q.8.h ; hainan haling chemipharma corporation , ltd . , haikou , china ) and careful nursing with electrocardiograph monitoring ( mp 30 ; philips medical systems b.v . ) for 10 days , the patient demonstrated clinical improvement ( abdominal pain was relieved , and the patient experienced no chills , nausea or vomiting , and vital signs were stable ) and was discharged .
after 5 days , the patient was readmitted due to high fever ( 39.2c ) , upper abdominal pain , nausea and vomiting , particularly after a fatty meal .
abdominal x - ray and ct scanning ( the somatom sensation 16 ; siemens ag , munich , germany ) revealed sporadic air - fluid levels and intestinal wall thickness ( fig .
2 ) . anti - infective treatment ( cefoperazone and sulbactam , both 1.5 g intravenous infection , q.12.h ; pfizer , inc . , new york , ny , usa ) was administered , but was ineffective .
multiple rashes on the lower extremities appeared on the fourth day , and laboratory examinations showed a rheumatoid factor of 20.4 iu / ml ( increased ) , antinuclear antibody 1:320 ( increased ) ; esr 26.00 mm / h ( increased ) ; anti - ribonucleoprotein antibody + + + ( strong ) , anti - smith antibody + + + ( strong ) , anti sjogren 's syndrome a antibody + + + ( strong ) and anti - sjogren 's syndrome b antibody + ( weak ) .
antibodies against double stranded - dna was not detected ; complement ( c3 ) , 0.39 g / l ( decreased ) ; crp , 7.0 mg / l ( normal ) ; wbc , 2.510/l ( decreased ) and hgb , 89 g / l ( decreased ) .
furthermore , the patient had experienced hair loss and multiple red rashes in response to strong sunlight the previous year .
sle and lupus - related ipso were then diagnosed according to american college of rheumatology criteria ( 1997 ) ( 8) , and the systemic lupus erythematosus disease activity index ( sledai ) score was 10 ( 8) .
the patient was treated with 2 mg / kg / day methylprednisolone ( pfizer , inc . ) and her general condition improved after 2 days . however , the patient had sudden onset of fever ( 40.4c ) and dyspnea , oxygen saturation ( sao2 ) was 87% , and arterial blood gas analysis revealed a ph of 7.409 , pco2 , 33.6 mmhg and po2 , 83.9 mmhg .
further laboratory examination showed : glutamic pyruvic transaminase , 436 u / l ( increased ) ; glutamic oxaloacetic transaminase , 740 u / l ( increased ) ; k , 3.36 mmol / l ( decreased ) ; and wbc , hgb , crp and urinalysis were normal . on chest ct
, both lungs showed ground - glass appearance , with uneven density , interlobular septal thickening and bilateral hydrothorax ( fig .
anti - infective treatment ( imipenem and cilastatin , 0.5 g intravenous injection , q.8.h ) was administered for 3 days ; however , fever and dyspnea were not alleviated .
a comprehensive infectious work - up ( blood , sputum and urine cultures ) was negative . a bronchoalveolar lavage ( bal ; olympus bx43 ; olympus corporation , tokyo , japan ) examination from the affected areas was also negative for gram positive / negative bacteria , viral infections , fungal infections , acid fast bacilli or hemosiderin - laden macrophage .
the erythematous rash was accentuated , and pathological biopsy results ( using h&e staining ) showed subcutaneous fibrous tissue hyperplasia with inflammatory cell infiltration ( fig . 3c and d ) .
alp was finally diagnosed and high - dose ( 1 g / day ) methylprednisolone + gamma globulin therapy was administered . after 3 days treatment ,
this therapy was followed by 2 mg / kg / day methylprednisolone as maintenance treatment , and glycyrrhizin ( minophagen pharmaceutical co. , ltd .
repeat chest ct scan showed significant improvement . during long - term tapering period or after stopping corticosteroids ( methylprednisolone , pfizer , inc . ) , the patient recovered well with no relapse detected .
the present study presents a rare case in which ipso was the initial presentation of sle with secondary onset of alp after emergency surgery . to the best of our knowledge
, there have been no previous reports of ipso and alp occurring in a single patient .
ipso is defined as the presence of clinical features suggesting intestinal obstruction , but without organic obstruction ( 9 ) . as in the present case ,
the clinical manifestations of ipso include abdominal pain , nausea , vomiting , absence of bowel sounds and may include constipation and fever ( 4 ) .
an x - ray and ct scan frequently show air - fluid levels and thickening of the small and/or large intestinal wall .
ipso may be the first manifestation of sle in some patients ; however , it usually represents a complication , with few case series described in the literature ( 1,2,10 ) .
ipso is easily confused with mechanical intestinal obstruction and represents a diagnostic challenge for the surgeon .
ureterohydronephrosis is highly associated with ipso in sle ( 1,4,10 ) , as repeated urinalysis examination results were negative , ureterohydronephrosis did not occur in this patient .
the pathophysiology of ipso secondary to sle remains unclear ( 9 ) , although the hypotheses have suggested dysmotility due to a primary myopathy or secondary to an immune complex mediated vasculitis ( 11 ) .
the pathological characteristics of the gastrointestinal tract in chronic intestinal pseudo - obstruction ( cipo ) patients include widespread myocyte necrosis , severe atrophy and fibrosis in the muscularis layer ; active serositis with serosal thickening and fibrosis , with little or no evidence of vasculitis or injury ( 11,12 ) . in the present aipo case ,
the pathological results of the jejunum showed muscle layer atrophy , with submucosal anapetia , congestion and hemorrhage .
therefore , the present case supports the hypothesis that the clinical features of aipo in sle patients appear to be the result of a primary intestinal smooth muscle myopathy , similar to that in cipo .
alp is an uncommon ( 112% ) manifestation , but may be a life - threatening complication of sle ( 12 ) .
the clinical features of alp are characterized by acute onset of fever , cough , pleurisy and dyspnea .
the key radiographic manifestation in the majority of cases is pulmonary infiltration , which appears on ct scan as ground - glass or honeycomb appearance ( 5,13 ) .
the diagnosis of alp is essential to exclude other causes of lung infiltration , particularly infective pneumonia ( 7 ) . in the present case
infective pneumonia was excluded , as a comprehensive infectious work - up was negative and anti - infective treatment was ineffective .
diffuse alveolar hemorrhage was excluded by the negative results of hemosiderin - laden macrophage in bal fluid .
alp was finally diagnosed by the syndrome of acute reversible hypoxemia , the abnormal results of ct scan , and the exclusion of other causes of lung infiltration .
the pathophysiology of alp is based on an acute injury to the alveolar capillary units in pulmonary vasculature ( 7 ) .
alp typically occurs during the active stage of sle , which may partially explain why mortality in alp is ~50% even when treated with large doses of steroids ( 14 ) .
emergency surgery has been shown to be an independent risk factor for increasing the complication rate and morbidity of sle patients ( 15,16 ) . in the present case , although the sledai score was 10 , alp occurred , which may be explained by the emergency surgery performed for ipso .
the trauma of abdominal operation enhances inflammatory function , which may have promoted the activity of autoimmunity in sle patients and eventually caused the occurrence of alp .
the patient 's clinical symptoms were ameliorated and the radiographic results showed improvement following high - dose methylprednisolone + gamma globulin supplement therapy for 3 days followed by 2 mg / kg methylprednisolone as maintenance treatment . in conclusion , we report the first case of alp and ipso co - existing in a patient with sle .
it remains a challenge for the surgeon to establish the correct diagnosis and prevent inappropriate surgical intervention when ipso plays a role in the initial presentation of sle .
alp typically occurs during the active stage of sle , thus emergency surgery may increase the rate of this complication .
 intestinal pseudo - obstruction ( ipso ) and acute lupus pneumonitis ( alp ) are uncommon severe complications of systemic lupus erythematosus ( sle ) . 
 the present study reports the case of a 26-year - old female who presented with abdominal pain , nausea and vomiting as initial symptoms . 
 computed tomography ( ct ) scanning revealed the jejunal wall was thickened and streaky , mimicking the presentation of intestinal obstruction . 
 following emergency surgery , the patient 's general condition was aggravated , with evident limb erythematous rashes . 
 a series of laboratory examinations revealed sle , and combined with patient 's medical history ipso was diagnosed , with a disease activity index score of 10 . during the therapeutic period , 
 high fever , dyspnea and oxygen saturation ( sao2 ) reductions were detected , and ct scans indicated lung infiltration , excluding other causes through a comprehensive infectious work - up and a bronchoalveolar lavage examination . 
 alp was confirmed and treated with high - dose methylprednisolone and gamma globulin supplement . 
 the patient responded well and was discharged in 2 weeks . in the one - year tapering period and after stopping corticosteroids , the patient recovered well with no relapse detected . in conclusion , 
 the manifestation of ipso in sle is rare and represents a challenge for the surgeon to establish the correct diagnosis and avoid inappropriate surgical intervention . 
 alp may be the consequence of emergency surgery , and immediate high - dose glucocorticoid therapy is recommended . 
Introduction
Case report
Discussion