Doença anti-membrana basal glomerular atípica: revisão de literatura

Abstract

Atypical Anti-Glomerular Basement Membrane (anti-GBM) antibody disease is an uncommon presentation of a rare disease with a more challenging diagnosis due to seronegativity and a more indolent progression. The clinical presentation is highly variable, with various types of glomerular disease manifestations such as hematuria, nephritic syndrome, nephrotic proteinuria, and loss of glomerular filtration rate, occurring with varying degrees of severity and temporality. Pulmonary hemorrhage is less common than in the classic presentation. Diagnosis requires renal biopsy showing linear deposits of immunoglobulin G in the glomerular basement membrane. Sixteen scientific articles from the PubMed database, published between 2019 and 2024, were selected, including fifteen case reports and one cohort study. Treatment involves aggressive immunosuppression with plasmapheresis, pulse therapy with corticosteroids, and immunosuppressants such as cyclophosphamide and Rituximab.