A35-22 Recurrent ANCA-Associated Vasculitis Presenting with Pulmonary-Renal Syndrome

Abstract Background Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, is a rare vasculitis that primarily affects the upper and lower respiratory tracts and the kidneys. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, such as GPA, can cause life-threatening pulmonary-renal syndrome characterized by diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Diffuse alveolar hemorrhage (DAH) occurs in approximately 5-15% of ANCA vasculitis cases and carries high mortality if not promptly treated. Relapses are common (approximately 60% within five years), necessitating vigilant long-term monitoring. Case Report We present a complex case of a 62-year-old male with a history of hypertension and chronic kidney disease (CKD), in addition to GPA. He presented with an acute relapse manifesting as pulmonary hemorrhage and renal impairment. A previous kidney biopsy had revealed necrotizing, crescentic, and sclerosing pauci-immune glomerulopathy, consistent with ANCA-associated crescentic glomerulonephritis. On admission, he was hypoxic, and his serum creatinine had acutely risen to 3.62 mg/dL, from baseline 2.7 to 2.8 mg/dl. Chest X-ray showed new bilateral infiltrates, and CT revealed diffuse ground-glass opacities consistent with DAH. Given his history and a recent high ANCA titer, a relapse of GPA was diagnosed, manifesting as his first episode of pulmonary-renal syndrome. He was treated with intravenous methylprednisolone, cyclophosphamide, and four sessions of plasmapheresis. The patient’s oxygenation and renal function improved (creatinine to 3.10 mg/dL), hemoptysis ceased, and he was discharged on a prednisone taper with strict nephrology and pulmonology follow-up. Discussion This case underscores the critical importance of recognizing rare complications such as diffuse alveolar hemorrhage and rapidly progressing glomerulonephritis, which carries a high mortality rate if not identified and intervened on, especially in patients presenting with hemoptysis, respiratory distress, and renal failure. Diffuse alveolar hemorrhage is a serious but potentially reversible manifestation of GPA and requires prompt immunosuppressive therapy. This patient’s acute presentation, coupled with renal dysfunction and chronic vasculitis, illustrates the systemic nature of the disease and the diagnostic challenges it presents in the acute care setting. Conclusion In patients with GPA, new or worsening respiratory symptoms and hemoptysis should raise concern for pulmonary vasculitis or alveolar hemorrhage. Prompt recognition and multidisciplinary involvement are crucial for definitive management and preventing delays in treatment that could lead to permanent or life-threatening complications. This abstract is funded by: none