A 28-year-old male patient, a heavy smoker with a body mass index of 24.8, with a history of hepatitis A 3 months before admission, and asthma since the age of 19, was admitted with one week of colicky periumbilical pain radiating to the lower abdomen and upper thighs. The pain gradually increased in intensity and was associated with distension, general fatigue, a fever of 39.1 C, anorexia, and unintentional weight loss (around 10 kg in one month). Also, he reported shortness of breath, lower limb swelling, and upper back and bilateral hand numbness that progressed to severe pain. There were no reports of trauma, heartburn, vomiting, diarrhoea, flatulence, melena, or haematochezia.
The patient was admitted to the intensive care unit at the age of 19 with exertional dyspnoea, orthopnoea, and lower limb oedema, according to his medical history. A diagnosis of pneumonia with atelectasis and delirium was made. He also had a history of diffuse nasal polyposis, grade 4, as well as an allergy to nonsteroidal anti-inflammatory drugs (NSAIDs) and penicillin. Additionally, at the age of 2, he underwent adenoidectomy and tonsillectomy procedures.
Chest examination revealed barrel shape, diminished expansion and faint wheezes. Additionally, hepatomegaly with a 20 cm liver span in the midclavicular line was found and confirmed by an ultrasound study. Both inguinal and cervical lymph nodes were palpable, bilaterally firm, rounded, nontender, and 1 to 2 cm each in size. Over the abdomen, audible bowel gurgling sounds were heard. Moreover, marked bilateral exophthalmos (Figure 1) and bilateral Raynaud's hands with bluish discolouration were observed (Figure 2). His right radial pulse was absent, whereas the left radial pulse was weak. On the other hand, the examination of the lower limbs was normal, and no discrepancies between arm and leg blood pressure were found.
Laboratory tests revealed anaemia and elevated levels of IgE and IgG, inflammatory markers, and the creatinine level. In addition, rheumatoid factor and antismooth muscle antibodies were positive (Table 1). However, electrolyte levels, liver enzymes, a thyroid function test, serological analysis, thrombophilia studies, an albumin blood test, and tumour biomarkers (PSA, CEA, and AFP) were all normal.
A computed tomography (CT) Pan-Scan showed mild bronchial wall thickening and centrilobular opacification. Hepatomegaly with an inhomogeneous mottled pattern of contrast enhancement was seen. Para-aortic, mediastinal, and hilar LNs were small, none exceeding 6 mm multiple small inguinal LNs were noted, none over 12 mm. Sinus CT without contrast revealed a paranasal sinus mucocele.
Three months after admission, laboratory tests showed 3+ proteinuria and 1.2 mg/dL creatinine. An echocardiogram revealed dilated left ventricle (6 cm), reduced systolic function, mild left ventricular hypertrophy, mildly dilated chambers, and moderate aortic regurgitation (interventricular septum thickness 1.2 cm, EF = 40%). As a result, heart failure therapy (enalapril, spironolactone, and carvedilol) was initiated.
An inguinal LN biopsy showed lymphoid follicles an increased number of eosinophils surrounding dilated and hyalinised blood vessels. No evidence of malignancy was found. Features were mostly consistent with Kimura's disease. A biopsy of the bone marrow revealed normocellular bone marrow with a granulomatous reaction and eosinophilic infiltration. The kidney biopsy revealed glomerular segmental sclerosis, and a diagnosis of focal segmental glomerulosclerosis was made. Therefore, moderate-dose steroids (tapered within a few weeks) and Azathioprine were added to his medications regimen.
Contrary to medical advice, he has been off medication throughout 2019. He developed a right round, firm, and nontender lump in his neck. The US revealed approximately 7 x 7 mm LN, located in the middle of the left lateral aspect of the neck with loss of the hilum, and a left submandibular gland of coarse echotexture (Figures 3 and 4). He also experienced paraesthesia in the right arm and right leg; electromyography indicated right tibial nerve axonal neuropathy. Moreover, he had several white, well-defined, and painless tongue ulcers (Figure 5) that were persistent over a year, oval in shape, less than 1 cm in size, more than 14 in number, and distributed along the anteromedial and the anterolateral portions of the ventral aspect of the tongue.
A year later, Mycophenolate Mofetil was started in place of Azathioprine since it was more effective in suppressing the disease and reducing GI distress. The patient's most recent examination revealed improved symptoms of asthma and cardiomyopathy. His last echo revealed normal LV size and function (nearly 55% ejection fraction), and serum IgG and IgE levels were within the normal range.