ABSTRACT
Microscopic polyangiitis is a systemic small-vessel vasculitis primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. This disease is determined by clinical manifestation, ANCA, and involved organ or renal biopsy. We describe a 49-year-old female presenting with chronic postnasal drip, progressive dyspnea and abrupt hemoptysis. Her serum p-ANCA was postitive, but c- ANCA was negative. Her chest X-ray and chest CT scan showed pulmonary hemorrhage, and the renal biopsy specimen revealed crescentic glomerulonephritis. She was diagnosed as having an ANCA-associated vasculitis, and more specifically, a microscopic polyangiitis accompanied with paranasal sinusitis. She was treated with intravenous methylprednisolone pulse therapy, followed by prednisolone and cyclophosphamide per oral tablets. She showed rapid progression of renal failure and died from sepsis after 2 months of treatment.