Anti-glomerular basement membrane vasculitis.
Anti-GBM disease is a rare, aggressive small-vessel vasculitis driven by autoantibodies against the alpha-3 chain of type IV collagen; prompt plasma exchange plus glucocorticoids and cyclophosphamide remains the standard of care, with kidney outcomes hinging on the degree of injury at presentation.
Background
Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune small-vessel vasculitis affecting the glomerular and pulmonary capillaries, caused by autoantibodies directed against the alpha-3 chain of type IV collagen. It typically presents as rapidly progressive glomerulonephritis, with concurrent pulmonary (alveolar) haemorrhage in roughly half of cases. Diagnosis rests on clinical features, kidney biopsy showing linear IgG deposition along the GBM, and detection of circulating anti-GBM antibodies.
Key recommendations
The introduction of plasma exchange combined with cyclophosphamide and glucocorticoids significantly improved outcomes over the historically near-fatal untreated course, particularly in patients who are not dialysis-dependent at presentation. Early initiation of this combination is crucial. Unlike ANCA-associated vasculitis, relapses are rare in classic anti-GBM disease and routine long-term maintenance immunosuppression is not required, although double-positive patients carrying both anti-GBM and ANCA have higher relapse risk and do need maintenance treatment.
Clinical implications
Dialysis dependency at presentation, a high proportion of glomerular crescents, and a low percentage of normal glomeruli are dominant predictors of poor kidney recovery, and treatment decisions in such cases require an individualized weighing of risks and benefits. Atypical and seronegative presentations are increasingly recognized but their optimal management remains unclear. Emerging therapies under investigation include rituximab and imlifidase (an IgG-cleaving agent), which may inform more individualized treatment in the future.
Category
Research
Source
Autoimmun Rev
More from ASNRT News
Browse the latest news, society announcements, KDIGO guideline updates, and AJNT issue releases on the ASNRT newsroom.