Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis

Abstract

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.

Keywords

• diffuse alveolar hemorrhage
• granulomatosis with polyangiitis
• plasmapheresis
• prednisone
• cyclophosphamide
• rituximab