Abstract Granulomatosis with polyangiitis (GPA) is a necrotizing small-vessel vasculitis classically involving the upper and lower respiratory tracts and kidneys. While pulmonary-renal syndrome is a recognized severe manifestation, presentation with gastrointestinal bleeding and fulminant respiratory failure is exceedingly rare. Early recognition is critical, as timely immunosuppressive therapy can be lifesaving. We report a case of GPA manifesting with massive gastrointestinal bleeding and catastrophic pulmonary-renal syndrome complicated by severe acute respiratory distress syndrome (ARDS) requiring veno-venous extracorporeal membrane oxygenation (VV-ECMO). A 45-year-old man with a history of asthma and prediabetes presented with hematemesis and melena following two months of intermittent vomiting and one month of dark stools, acutely worsening over two days. On arrival, he was tachycardic and profoundly hypoxic (SpO2 70% on room air). Laboratory evaluation revealed severe anemia (Hgb 5.7 g/dL), marked azotemia (BUN 130 mg/dL, Cr 19.26 mg/dL), metabolic acidosis (HCO3 11 mmol/L, anion gap 18), and hyperkalemia. Chest radiograph showed diffuse bilateral airspace opacities concerning for pneumonia, and broad-spectrum antibiotics were initiated. The patient’s condition rapidly deteriorated, developing acute hypoxemic respiratory failure and oliguric renal failure requiring intubation, vasopressor support, and initiation of renal replacement therapy. Despite maximal ventilatory support, severe ARDS ensued, refractory to high PEEP and FiO2, necessitating rescue VV-ECMO. Given the concurrent respiratory and renal failure, high-dose intravenous methylprednisolone was initiated for presumed autoimmune vasculitis. Serologic testing returned positive for cytoplasmic ANCA (c-ANCA) and anti-proteinase 3 antibodies. Subsequent renal biopsy revealed chronic crescentic pauci-immune glomerulonephritis with fibrosis and crescent formation, confirming ANCA-associated vasculitis. The patient was diagnosed with GPA presenting as pulmonary-renal syndrome and treated with rituximab and a corticosteroid taper. Over several weeks, he demonstrated remarkable recovery in both renal and pulmonary function, was successfully decannulated from ECMO, and later discharged to rehabilitation. This case highlights an extraordinary presentation of GPA manifesting as gastrointestinal bleeding with life-threatening pulmonary-renal involvement. The overlap of diffuse alveolar hemorrhage and ARDS can obscure the underlying vasculitic process, delaying immunosuppressive therapy. Clinicians should maintain a high index of suspicion for ANCA-associated vasculitis in patients presenting with unexplained pulmonary infiltrates, renal failure, and anemia. Early recognition, prompt initiation of immunosuppression, and multidisciplinary coordination, including ECMO as a bridge to recovery, can significantly improve survival in catastrophic presentations of systemic vasculitis. This abstract is funded by: None
Chaney et al. (Fri,) studied this question.