B53-14 When Hemoptysis Meets Renal Failure: Navigating the Complexities of Granulomatosis Polyangiitis in a Critical Patient

Abstract Introduction Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are characterized by necrotizing inflammation of small- and medium-sized vessels. Granulomatosis with polyangiitis (GPA), a proteinase 3 (PR 3) ANCA, is a rare disease with a prevalence of 0.042% in the United States and typically involves the lungs and kidneys. Description A 60-year-old Jehovah’s Witness male with celiac disease, newly diagnosed ANCA-associated vasculitis, prior Mycobacterium avium-intracellulare (MAC), Pneumocystis jirovecii (PJP), and Aspergillus infections, hypogammaglobinemia, and a history of deep venous thrombus (not on anticoagulation) presented with acute hypoxemic respiratory failure secondary to suspected diffuse alveolar hemorrhage (DAH). He experienced recurrent hemoptysis for the past 8 months and previously underwent suction tamponade, desmopressin, tranexamic acid, and right bronchial artery embolization without sustained improvement.Workup during a prior hospitalization was notable for positive PR3-ANCA and renal involvement suggestive of GPA. He received corticosteroids and rituximab; however, his symptoms worsened, requiring multiple readmissions for recurrent hemoptysis. On this current admission, he presented with hemoptysis, arthralgias, and myalgias. His condition rapidly deteriorated with massive hemoptysis leading to hypoxemic respiratory failure, intubation, and ICU transfer. His hospital course was complicated by shock, requiring pressors, acute anemia, and progressive renal failure requiring hemodialysis. GPA was managed with pulse dose steroids and rituximab, but plasma exchange and immunoglobulin therapy were declined in accordance with his religious beliefs. Despite aggressive medical management, his respiratory and renal failure progressed. After multidisciplinary discussions, he elected for comfort measures only and, unfortunately, expired. Discussion This case highlights both ethical complexities with managing life-threatening complications of GPA in a Jehovah’s Witness and the therapeutic challenge of managing infections while in an active GPA flare. Optimal immunosuppressive regimens in the setting of active infections remain an area for future research. This abstract is funded by: None