Monotypic IgG1-kappa Atypical Anti-Glomerular Basement Membrane Nephritis: A Case Report

Autor: Danielle Lee, Viresh Mohanlal, Harold Watson, Steven Carlan, Mario Madruga, Maxim Olivier
Rok vydání: 2019
Předmět:
Zdroj: Case Reports in Nephrology and Dialysis, Vol 9, Iss 1, Pp 8-14 (2019)
Case Reports in Nephrology and Dialysis
ISSN: 2296-9705
DOI: 10.1159/000498844
Popis: Anti-glomerular basement membrane (anti-GBM) glomerulonephritis is a rare disease caused by autoantibodies against the glomerular basement membrane. Atypical anti-GBM nephritis is clinically less aggressive and characterized by the absence of circulating autoantibodies to the basement membrane. A previously healthy 53-year-old white woman presented with a rising creatinine over a short observation period. Renal biopsy, urinary sediment, and laboratory testing confirmed the diagnosis of atypical anti-GBM disease. She received plasmapheresis, steroids, and cyclophosphamide. She developed hemorrhagic cystitis early in the treatment from oral cyclophosphamide and mycophenolate mofetil was substituted as a first-line drug. She responded favorably and continued on mycophenolate mofetil without evidence of relapse. Despite the absence of circulating autoantibodies, a diagnosis of atypical anti-GBM nephritis should not be excluded if a high index of clinical suspicion exists. Early renal biopsy should be considered. Mycophenolate mofetil may be a reasonable replacement for oral cyclophosphamide in the treatment of atypical anti-GBM disease when cyclophosphamide is contraindicated.
Databáze: OpenAIRE
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