Churg-Strauss syndrome (CSS) also called allergic granulomatosis and angiitis is a multisystem disorder. Churg-Strauss syndrome is defined as an eosinophil-rich, granulomatous inflammation involving the respiratory tract, along with necrotizing vasculitis affecting small- to medium-sized vessels, and is associated with asthma and eosinophilia. Renal involvement in CSS varies from 26 to 88 % but is usually of mild to moderate stage, and advanced renal failure is uncommon. We encountered an unusual case of 27-year-old man with asthma and primary focal segmental glomerulosclerosis diagnosed as CSS showing myeloperoxidase anti-neutrophil cytoplasmic antibody-associated acute kidney injury with crescentic glomerulonephritis. Patient responded to steroid and cyclophosphamide. Over a follow-up of 2 months, he has no hematuria/eosinophilia and serum creatinine of 2.3 mg/dL has decreased to 1.7 mg/dL.
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Department of Otorhinolaryngology-Head and Neck Surgery, Graduate School of Medicine, The University of Tokyo.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a disease with marked elevation of blood eosinophil associated with various symptoms in the respiratory tract, gastrointestinal tract, peripheral nerves, and skin due to systemic vasculitis. Here, we report a case of EGPA in which rhinosinusitis was treated with endoscopic sinus surgery (ESS) with good results. A 49-year-old man who developed bronchial asthma at age 48 was diagnosed with EGPA in our hospital.
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