C41-36 From Cough to ECMO: Fulminant Granulomatosis With Polyangiitis Presenting as Diffuse Alveolar Hemorrhage

Abstract Introduction Granulomatosis with polyangiitis (GPA) is a necrotizing small-vessel vasculitis characterized by granulomatous inflammation primarily involving the respiratory tract and kidneys. Pulmonary-renal syndrome is a well-recognized but potentially fatal manifestation. Diffuse alveolar hemorrhage (DAH) causing respiratory failure severe enough to require extracorporeal membrane oxygenation (ECMO) remains rare and represents one of the most critical presentations of GPA. Case A 51-year-old man with hypertension, chronic sinusitis, and recent significant weight loss presented with two days of worsening shortness of breath, chest tightness, and new-onset hemoptysis. He also reported fatigue and bilateral leg swelling for two weeks. Initial evaluation revealed acute renal failure and diffuse pulmonary infiltrates. Laboratory testing showed a positive c-ANCA titer of 1:160, consistent with granulomatosis with polyangiitis. Computed tomography of the chest demonstrated diffuse alveolar infiltrates without pulmonary embolism, which progressed to extensive bilateral ground-glass opacities and consolidation. Despite noninvasive support, the patient developed severe hypoxemic respiratory failure requiring intubation, paralysis, and inhaled nitric oxide. Bronchoscopy confirmed diffuse alveolar hemorrhage. Due to refractory hypoxemia, ECMO was initiated for rescue therapy. High-dose intravenous methylprednisolone was administered for three days, followed by maintenance corticosteroids. Cyclophosphamide and plasmapheresis were initiated, with plans for rituximab. The patient also required intermittent hemodialysis for worsening renal function. Discussion and Conclusion This case demonstrates the fulminant course of GPA manifesting as pulmonary-renal syndrome with life-threatening diffuse alveolar hemorrhage. The underlying necrotizing vasculitis leads to capillaritis-induced alveolar bleeding and glomerular injury, resulting in rapid multiorgan failure. Early recognition and initiation of immunosuppressive therapy are essential, but severe cases may require advanced support such as ECMO when conventional ventilation fails. This case underscores the importance of prompt diagnosis and aggressive management in preventing mortality from this rare but devastating presentation of GPA. This abstract is funded by: None