Abstract Granulomatosis with polyangiitis (GPA) is a rare, systemic, necrotizing vasculitis affecting small and medium-sized vessels, mainly involving the respiratory tract and kidneys. It is classified as an ANCA-associated vasculitis (AAV), with severe presentations including diffuse alveolar hemorrhage (DAH) and rapidly progressive glomerulonephritis, together called pulmonary-renal syndrome. Such cases can result in life-threatening respiratory and renal failure requiring advanced organ support. A 33-year-old man with no significant past medical history presented with cough, hemoptysis, and bilateral lower extremity edema. Chest imaging showed multifocal pulmonary infiltrates, and urinalysis revealed microscopic hematuria with preserved renal function. Empiric antibiotics and diuretics for suspected pneumonia and heart failure were initiated. Despite therapy, he developed worsening hypoxemia requiring intubation. Bronchoscopy confirmed DAH. Autoimmune testing revealed positive C-ANCA (1:1280 speckled) and PR3-ANCA 115, consistent with GPA. High-dose steroids and plasma exchange (PLEX) were initiated. His course was complicated by acute kidney injury progressing to anuria and azotemia, necessitating continuous renal replacement therapy (CRRT). On hospital day six, refractory respiratory failure led to initiation of veno-venous extracorporeal membrane oxygenation (VV ECMO). The patient received seven PLEX sessions, rituximab, and a 5-day steroid pulse with taper. He was successfully weaned from ECMO and CRRT, underwent tracheostomy and PEG placement for prolonged support, and achieved full recovery with subsequent decannulation. Management of severe GPA with pulmonary-renal syndrome remains challenging, particularly when organ failure necessitates advanced support. Current guidelines recommend high-dose glucocorticoids and either rituximab or cyclophosphamide for induction. ECMO provides effective oxygenation in refractory DAH, allowing time for immunosuppressive therapy to achieve disease control while minimizing ventilator induced lung injury. CRRT supports renal recovery and fluid balance and can be safely integrated into ECMO circuits. This case highlights the successful use of ECMO and CRRT in GPA induced pulmonary-renal syndrome. With multidisciplinary coordination and individualized anticoagulation, these modalities can be lifesaving and allow time for definitive immunosuppressive therapy to achieve disease control. Advanced organ support should be considered in similar patients with refractory respiratory and renal failure. This abstract is funded by: None
Deal et al. (Fri,) studied this question.