Abstract: SA-PO767
Rituximab Induction in Rheumatoid-Associated, ANCA-Negative, Pauci-Immune Glomerulonephritis
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
- Song, Chunzi, The University of Texas Southwestern Medical Center, Dallas, Texas, United States
- Khayat, Maurice I., The University of Texas Southwestern Medical Center, Dallas, Texas, United States
Introduction
Rheumatoid vasculitis (RV) is a serious complication of rheumatoid arthritis (RA). Adoption of disease modifying antirheumatic drugs (DMARDs) has decreased its incidence. Renal involvement is rare, but pauci-immune glomerulonephritis (GN) with detectable antineutrophil cytoplasmic antibodies (ANCA) has been reported. The optimal therapy for ANCA-negative RV is unknown. Rituximab (RTX) is frequently used to treat articular symptoms, but no cases using RTX for ANCA-negative RV are described.
Case Description
A 68 year-old man with a history of RA presented with anasarca, acute kidney injury and nephrotic syndrome. His RA was diagnosed 15 years prior with erosive arthritis, positive antinuclear antibodies, rheumatoid factor, and anti-cyclic citrullinated peptide antibodies. He was started on methotrexate, but after 5 years he was lost to follow up.
On re-presentation after 10 years, labs were notable for a serum creatinine between 2.6-3.0 mg/dL, albumin of <1 mg/dL, and urine protein-to-creatinine ratio between 5.4-14 g/g. Renal biopsy revealed pauci-immune focal crescentic GN, global and segmental glomerulosclerosis without interstitial fibrosis or tubular atrophy (IFTA). Congo red stain was negative. Serologic work up including serum ANCA was negative except as outlined above. No extrarenal manifestations of vasculitis were noted.
RTX 1000mg, 2 weeks apart resulted in reduction of proteinuria with unchanged serum creatinine and albumin. Repeat renal biopsy after 3 months revealed resolution of crescentic vasculitis, development of moderate IFTA and focal segmental glomerulosclerosis (FSGS). Complications included hypervolemia requiring temporary renal replacement therapy and persistent nephrotic syndrome. A short course of empiric corticosteroids was stopped due to disease quiescence following biopsy results.
Discussion
ANCA-negative RV with crescentic GN is rare, and the optimal treatment is uncertain. Of the three described cases, all received corticosteroids, two were given cyclophosphamide, and one was treated with plasma exchange. To our knowledge, this is the first reported case utiliizing RTX induction. For our patient, rituximab resulted in remission of active crescentic disease, but not the development of chronic sequelae. While RTX remains an attractive potential treatment option for ANCA-negative RV, further investigation is warranted.