Anti-glomerular basement membrane disease-treatment standard.
A 2025 practice review from the European Renal Association outlines current anti-GBM disease treatment standards - plasma exchange, cyclophosphamide, and steroids - while highlighting open questions about rituximab and emerging agents like imlifidase.
Background
Anti-GBM disease is caused by autoantibodies against the alpha3 chain of type IV collagen, producing rapidly progressive glomerulonephritis with pulmonary hemorrhage in about half of cases; before plasma exchange became standard, the disease was typically fatal.
Key findings
The combination of plasma exchange, cyclophosphamide, and glucocorticoids has substantially improved outcomes, particularly in patients who are not yet dialysis-dependent at presentation. Relapses are rare in classic anti-GBM disease, but 'double-positive' patients with concurrent ANCA positivity have a higher relapse risk and need maintenance immunosuppression.
Clinical implications
Treatment decisions should weigh biopsy findings, dialysis status, and immunosuppression contraindications; the authors call for trials clarifying the role of rituximab and emerging agents like imlifidase, and whether oral cyclophosphamide can replace intravenous dosing.
Category
Transplant
Source
Nephrology Dialysis Transplantation
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