Síndrome pulmón-riñón, hemorragia pulmonar difusa y glomerulonefritis rápidamente progresiva asociada a granulomatosis de Wegener, caso clínico y revisión corta

Abstract

We report the case of a 41-year-old Mexican woman with systemic small vessel vasculitis affecting the kidney with pauci-immune rapidly progressive glomerulonephritis and diffuse pulmonary hemorrhage. This life-threatening condition warrants immediate intensive treatment with combination therapy with methylprednisolone, cyclophosphamide, and plasma exchange. The patient showed a transient improvement, with subsequent progressive deterioration. The results of renal biopsy showed high activity after treatment. B-cell lymphocyte depletion therapy (rituximab) was started, producing substantial clinical and histologic improvement, with complete remission.