Epidemiology, clinical features, risk factors, and outcomes in anti-glomerular basement membrane disease: A systematic review and meta-analysis.
A meta-analysis of 47 studies and nearly 2,900 patients with anti-GBM disease found that while roughly three-quarters of patients survive the first year, kidney recovery remains poor, with only about 30% retaining kidney function at one year.
Background
Anti-glomerular basement membrane (anti-GBM) disease is a rare, aggressive small-vessel vasculitis caused by autoantibodies against type IV collagen in the glomerular and pulmonary capillaries, historically carrying a very poor prognosis without treatment.
Key findings
Pooling 47 studies and 2,830 patients, the authors found an annual incidence of 0.60-1.79 cases per million, with anti-GBM antibodies detected in 88.8% of cases, ANCA co-positivity in 27.4%, and pulmonary hemorrhage in 32.6%. One-year patient survival was 76.2%, but one-year kidney survival was only 30.2%.
Clinical implications
Kidney function at diagnosis and the percentage of normal glomeruli on biopsy were the strongest predictors of renal outcome, reinforcing the need for rapid diagnosis and treatment initiation before irreversible glomerular damage occurs.
Category
Research
Source
Autoimmunity Reviews
More from ASNRT News
Browse the latest news, society announcements, KDIGO guideline updates, and AJNT issue releases on the ASNRT newsroom.