Immunoglobulin G (IgG) deposits in the glomerular basement membrane (GBM) are linear in anti-glomerular basement membrane (anti-GBM) illness, which is characterized by crescentic necrotizing glomerulonephritis. Clinically, fast progressing glomerulonephritis with or without pulmonary bleeding is a feature of classic anti-GBM illness. Atypical anti-GBM illness is a subset of anti-GBM disease that has milder symptoms and a better prognosis for the kidneys than conventional anti-GBM disease. The capillary beds of the kidneys and lungs are affected by the small vessel vasculitis known as anti-GBM disease. Although the triggers that set off the autoimmune response are not fully understood, it is an autoimmune illness brought on by the formation of directly pathogenic autoantibodies that target a well-characterized autoantigen produced in the basement membranes of these organs. The confirmation of spatial and temporal clustering of cases in recent years raises the possibility that environmental variables, including infection, can cause disease in people with a genetic predisposition. 40% to 60% of patients will also experience concurrent alveolar bleeding as they develop broad glomerular crescent formation, which typically presents with signs of fast progressing GN. Rapid removal of pathogenic autoantibody is the goal of treatment, which frequently involves plasma exchange, steroids, and cytotoxic therapy to stop continued autoantibody formation and tissue inflammation. However, patients who present with oligoanuria, a high percentage of glomerular crescents, or kidney failure requiring dialysis have a poor prognosis for renal function. Retrospective cohort studies suggest that when this combination of treatments is initiated early, the majority of patients will have good renal outcomes. Although de novo anti-GBM disease after transplant for Alport syndrome is a reported event, relapse and recurring disease after kidney transplantation are both infrequent. Atypical anti-GBM illness presentations are also more frequently observed, and co-presentation with other kidney disorders such ANCA-associated vasculitis and membranous nephropathy seems to occur more frequently than would be predicted by chance alone. These findings emphasize the need for further research to clarify the immunopathogenic mechanisms behind anti-GBM illness and how to more effectively refine and enhance treatments, especially for patients who present with poor prognostic indicators.

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