The Churg-Strauss syndrome: An unusual presentation
A 20-year-old male presented to our institution with history of recurrent rhinosinusitis and bronchial asthma since 2007. He had malaise, anorexia, and weight loss of 10 kg in 6 months. He received antibiotics for his upper respiratory symptoms and underwent functional endoscopic sinus surgery with adenoidectomy and submucosal resection in August 2010. He was found to have mild renal failure in February 2011, when he was evaluated at another center for respiratory tract infection. On examination, he had frontal sinus tenderness; clinical examination was otherwise normal. Investigations showed total leukocyte count 20,500 cu/mm, Hb 12.5 g/dL, platelet count 341,000 cu/mm, peripheral blood smear: normal, serum creatinine 2.48 mg/dL, blood urea 89.7 mg/dL, serum electrolytes and liver function test: normal, urine protein ++, microscopy 5-10 RBCs/HPF. Throat swab culture: normal flora of upper respiratory tract. X-ray chest showed a few bilateral nonhomogenous opacities. ECG, echocardiogram, and ultrasonogram of abdomen were normal. Serum complements (C3 and C4) were normal. P-antineutrophil cytoplasmic antibodies (P-ANCA), C-ANCA, and antinuclear antibody (ANA) were negative. computerized tomographic scan of paranasal sinuses (plain) showed mild mucosal thickening in bilateral maxillary, bilateral ethmoidal, and bilateral frontal sinuses, suggestive of sinusitis. Kidney biopsy showed glomeruli with cellular crescents and many showed granulomatous response around the glomeruli. Many of the crescents [ and ] were seen to extend beyond the Bowman's capsule into the interstitium. The capillary tuft showed fibrinoid necrosis. The tubules showed focal nuclear loss, loss of brush border, and simplification. Tubular lumina showed occasional hyaline and WBC casts and tubular atrophy. The interstitium showed patchy edema, moderate to dense mixed inflammatory infiltrate composed of lymphocytes, plasma cells, neutrophils, and numerous eosinophils []. Ear, nose, and throat specialist opinion was sought; diagnostic nasal endoscopy was performed that showed features suggestive of chronic sinusitis. On ophthalmology evaluation, fluorescein angiography of the right eye showed mild vitreous haze superiorly, leakage around the vessels superiorly, and fuzziness of the vasculature in the superotemporal arcade, signifying vasculitis of retina [].\nIn view of history of asthma, features of sinusitis, pulmonary opacities detected radiographically, and kidney biopsy showing fibrinoid necrosis in glomeruli, granuloma, and eosinophils in extravascular areas, a diagnosis of CSS was made. Our patient had four of the six criteria proposed by the American College of Rheumatology for diagnosis of CSS. In our patient, peripheral blood eosinophilia and mononeuritis multiplex were not seen. He was started on prednisolone 1 mg/kg/day and cyclophosphamide 2 mg/kg/day. His serum creatinine level improved during the hospital stay. On follow-up, he was asymptomatic, his renal function improved, and findings on chest X-ray resolved.