Abstract: SA-PO764
Efficacy and Safety of Rituximab in Anti-glomerular Basement Membrane (GBM) Disease
Session Information
- ANCA-Associated Vasculitis, Anti-GBM Disease, and Other RPGN
October 26, 2024 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Ivkovic, Vanja, UHC Zagreb, Department of Nephrology, Hypertension, Dialysis and Transplantation, Zagreb, Zagreb, Croatia
- Bajema, Ingeborg M., Department of Pathology and Medical Biology, University of Groningen, University Medical Center, Groningen, Groningen, Netherlands
- Bruchfeld, Annette, Department of Health, Medicine and Caring Sciences, Linköping University, Linköping, Sweden
- McAdoo, Stephen Paul, Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, Hammersmith Campus, London, United Kingdom
- Segelmark, Marten, Department of Clinical Sciences, Lund University, Lund, Sweden
- Kronbichler, Andreas, Department of Internal Medicine IV, Nephrology and Hypertension, Medical University Innsbruck,, Innsbruck, Austria
Background
Anti-glomerular basement membrane (GBM) disease is a vasculitis caused by pathogenic antibodies targeting the NC1 domain of the α3 chain of type IV collagen and presenting as RPGN and diffuse alveolar haemorrhage (DAH). Decreasing production of these antibodies and their clearance are imperative to achieve patient and kidney survival. The mainstay of therapy is the combination of plasma exchange (PLEX), glucocorticoids and cyclophosphamide. Rituximab, an anti-CD20 monoclonal antibody causing B-cell depletion, has been proposed as a potential treatment for anti-GBM disease, but there is still a paucity of evidence of its efficacy and safety.
Methods
We have performed a systematic review of case reports/series providing individual patient-level data on the efficacy and safety of rituximab in anti-GBM disease. A search strategy of studies indexed in PubMed was performed followed by synthesis and analysis of the collated data.
Results
Twenty-two studies with data on 43 patients (M:F=18:15; median age 34 years, IQR: 20-63) were included who received rituximab in the first (N=18) or second line (N=25). Median serum creatinine was 416 µmol/L (IQR 212-706) with 21 (49%) patients being dialysis-dependent on presentation and 19 (44%) having DAH. Ten patients were positive for ANCA, 8 of them for MPO-ANCA. Almost all patients received pulse and oral glucocorticoids and PLEX (88%, 100% and 95%, respectively) and 62% of them received cyclophosphamide. Patients received a median of 4 (2-4) doses of rituximab with 5 patients (12%) having adverse effects which were all transient. After a median of 14.8 (5.0-33.0) months follow-up, patient survival was 100% and kidney survival 63% (33% in initially dialysis-dependent patients vs. 90% in initially non-dialysis-dependent patients, p<0.001). There were no differences in kidney survival between ANCA-positive and negative patients, those with and without DAH or those who received rituximab in first or second line.
Conclusion
In this, the largest group of anti-GBM disease patients receiving rituximab to date, the treatment had a favourable toxicity and efficacy profile. However, there is a great risk of publication bias and prospective trials are necessary to determine if rituximab should replace cyclophosphamide or be used in conjunction with cyclophosphamide as first line treatment.