Relapse of subacute anti-glomerular basement membrane nephritis following self-discontinuation of steroid therapy - PubMed
6 hours ago
- #Relapse
- #Anti-GBM nephritis
- #Hemodialysis
- A 23-year-old woman with no prior medical history was referred for proteinuria and elevated serum creatinine (Cr) level of 3.3 mg/dL.
- Kidney biopsy revealed crescentic glomerulonephritis with advanced glomerular sclerosis and diffuse linear staining of IgG along the GBM.
- She received corticosteroids, intravenous cyclophosphamide, and 7 sessions of plasma-exchanges, leading to improvement in Cr to 1.9 mg/dL and undetectable anti-GBM antibody.
- She self-discontinued prednisolone 2 months later, leading to relapse with Cr rising to 9.6 mg/dL and anti-GBM antibody titer at 4.0 U/mL.
- A second kidney biopsy showed progression of glomerulosclerosis, endothelial injury, and extensive tubulointerstitial damage.
- Despite resuming treatment, her kidney function did not recover, requiring maintenance hemodialysis.
- This case highlights atypical features of anti-GBM nephritis: subacute progression over 5 months and relapse after antibody negativity triggered by steroid discontinuation.
- Emphasizes the importance of adherence to immunosuppressive therapy, even after serological remission.