A Rare Case of IgA Vasculitis and Crescentic Glomerulonephritis in a Patient with Cirrhosis

Introduction: Rapidly progressing glomerulonephritits (RPGN), also known as crescentic glomerulonephritis, is a condition characterized by rapid decline in kidney function over days to weeks. RPGN is characterized by the presence of glomerular crescents seen on kidney biopsy. While most cases of RPGN are pauci-immune glomerulonephritis, less common causes must also be considered. We present an unusual case of RPGN from IgA vasculitis associated with cirrhosis. Case Description: A 37-year-old man with cirrhosis from alcohol use disorder presented to the emergency department with thrombocytopenia, anemia, and creatinine elevation to 6.1 mg/dL (from a prior baseline of 0.7). Urinalysis demonstrated +4 proteinuria and 3+ hematuria. Of note, he had been diagnosed with leukocytoclastic vasculitis with IgA deposits 4 months prior. On this current admission, his rash had worsened (image 1). He was given pulse dose steroids and renal biopsy was performed, which demonstrated diffuse mesangial and focal endocapillary proliferative and sclerosing glomerulonephritis with membranoproliferative features, focal crescents, and focal necrotizing features. Immunofluorescence demonstrated IgA dominant staining, confirming the diagnosis of crescentic IgA vasculitis. He was treated with cyclophosphamide for RPGN, and his creatinine stabilized. Discussion: Though IgA nephropathy is the most common glomerulonephritis, crescentic IgA and IgA vasculitis are relatively uncommon. Most patients with cirrhosis who are found to have IgA nephropathy have a mild, secondary form of IgAN. Our patient, in contrast, had extremely severe features of the spectrum of IgA related disorders, including both generalized IgA vasculitis and RPGN from IgAN. In a patient with cirrhosis and acute kidney injury, glomerulonephritis remains on the differential and may be responsive to immunosuppression.