Anti-GBM-Nephritis

Autoantibodies against the glomerular basement membrane (GBM) cause an aggressive form of small vessel vasculitis, previously also referred to as Goodpasture syndrome. The disease either runs a limited course in the kidneys (anti-GBM nephritis) or manifests as pulmonary renal syndrome (anti-GBM disease). Over the past decade, a broader range of manifestations has been recognized. In particular, double positive serology, i.e., the coexistence of anti-GBM autoantibodies and antineutrophil cytoplasmic antibodies (ANCA), has been delineated in more detail, which also has treatment implications. Urgent treatment is essential to decisively improve outcomes. Plasma exchange remains a central component of current treatment strategies, with the overall aim to remove pathogenic autoantibodies. Better clinical and histological markers have now emerged, enabling an early stratification of patients who will benefit from continuous immunosuppressive therapy in terms of the renal prognosis. This article provides an overview of novel insights into the disease course (including atypical variants), with a focus on novel clinically relevant aspects in the diagnostics and particularly in new treatment approaches. Imlifidase has emerged as a promising extension to the treatment options.