Abstract

Introduction: Rapidly progressive glomerulonephritis (RPGN) and widespread alveolar hemorrhage define ANCA-associated vasculitis (AAV), a rare, life-threatening illness (DAH).
 Case Presentation: An elderly female came with lower limb weakness and oliguria had features suggestive of RPRF and fluid overload. She developed hemoptysis with respiratory failure despite hemodialysis and intravenous steroids. The diagnosis of patients was pulmonary-renal syndrome– DAH in the setting of ANCA and based on the HRCT chest and positive p-ANCA report. She had excellent responses to intravenous pulse steroids, cyclophosphamide, and plasma exchange. 
 Conclusion: Based on observation showed the importance of immediate intervention in potentially fatal disease DAH in AAV.

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