Abstract Introduction IgA vasculitis, which typically presents as a benign course among children, can manifest with severe systemic complications in adults. We present a case of fulminant IgA vasculitis causing pulmonary-renal syndrome. Case Description A 38-year-old male with a past medical history of fatty liver disease and heavy alcohol use presented to the emergency department for generalized abdominal pain and diarrhea for the past two days. Laboratory data demonstrated leukocytosis, lactic acidosis, and acute kidney injury. Intravenous antibiotics were initiated for suspected enteritis and urinary tract infection. After the second day, the patient developed petechiae in the bilateral lower extremities. The hospital course was complicated by worsening renal function requiring initiation of hemodialysis. Renal biopsy was performed and was consistent with IgA glomerulonephritis with MEST/Oxford score: M1, E1, S1, T1, C0. The patient later developed hemoptysis and acute hypoxemic respiratory failure. CT was performed of the chest, abdomen, and pelvis, which was significant for cirrhosis, portal hypertension, and diffuse bilateral ground glass opacities. Bronchoscopy later confirmed diffuse alveolar hemorrhage. Due to worsening respiratory failure, the patient was subsequently intubated. For suspected IgA vasculitis induced pulmonary-renal syndrome, treatment was initiated with plasma exchange, IVIG, and high dose steroids. Additional rheumatological and microbiologic studies were unremarkable. Despite the above interventions, the patient’s condition failed to improve, and rituximab was initiated. Due to the persistent need for hemodialysis and rising MELD score, the decision was made to proceed with simultaneous kidney and liver transplantation. The patient was successfully extubated seven days post-operation and was weaned off hemodialysis ten days post-operation. Discussion IgA vasculitis is commonly characterized as a self-limiting, pediatric disease. This case demonstrates a rare presentation of IgA vasculitis, as it involved an adult who then developed life-threatening pulmonary-renal syndrome requiring dual organ transplantation. The atypical demographic and non-specific symptoms prior to developing the characteristic rash posed a challenge to a quick diagnosis. This case highlights how, once diagnosed, adult IgA vasculitis may demand a different clinical approach compared to the pediatric form and the importance of early multidisciplinary collaboration in optimizing patient outcomes. This abstract is funded by: None
Go et al. (Fri,) studied this question.