Kidney Week

Abstract: FR-PO0656

ANCA-Negative Relapses in Patients with ANCA-Associated Vasculitis

Session Information

• Glomerular Diseases: Clinical, Outcomes, and Therapeutics Research - ANCA/FSGS
  October 23, 2026 | Location: Exhibit Hall A, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Glomerular Diseases

• 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics

Authors

• Aaron, Sydney, Massachusetts General Hospital, Boston, Massachusetts, United States

Sydney Aaron

• Efe, Orhan, Massachusetts General Hospital, Boston, Massachusetts, United States

Orhan Efe, MD

• Al Jurdi, Ayman, Massachusetts General Hospital, Boston, Massachusetts, United States

Ayman Al Jurdi, MD, MPH, FASN

• Seethapathy, Harish Shanthanu, Massachusetts General Hospital, Boston, Massachusetts, United States

Harish Shanthanu Seethapathy, MD, MBBS

• Chadha, Ashima, Massachusetts General Hospital, Boston, Massachusetts, United States

Ashima Chadha, MD

• Chung, James L., Massachusetts General Hospital, Boston, Massachusetts, United States

James L. Chung, MA

• Laliberte, Karen A., Massachusetts General Hospital, Boston, Massachusetts, United States

Karen A. Laliberte, RN

• Niles, John L., Massachusetts General Hospital, Boston, Massachusetts, United States

John L. Niles, MD

Background

Relapses of ANCA vasculitis are typically associated with the presence of B cells and rising ANCA titers. Definite relapses with highly characteristic features combined with negative ANCA are rare.

Methods

Out of the 1095 patients in our clinic treated for ANCA vasculitis with rituximab and who have had more than one year of follow-up, we identified and characterized 8 patients who were diagnosed with relapse with negative serum ANCA.

Results

All 8 patients were on maintenance treatment at time of relapse (Table 1). 6 of 8 patients were treated with rituximab induction and maintenance. 4 of 8 were also B cell depleted at the time of relapse. 3 of 8 patients had at least one additional ANCA negative relapse with one patient having chronic activity while remaining ANCA negative and B cell depleted. Median time from diagnosis to relapse was 8.6 (5.6-11.0) years.
All 8 patients had characteristic features of GPA at initial disease and at relapse. Features at relapse included: scleritis 3/8, rhinitis and bloody crusting 2/8, acute otitis 5/8, tracheal stenosis 1/8 (Table 1). None of the patients had renal involvement at relapse.
After the relapse, all were treated with gentle reinduction immunosuppression including modest steroids and responded easily. 2 were subsequently well controlled on avacopan, 2 others on methotrexate monotherapy. 4 others were controlled with rituximab, and/or MMF 1, AZA 1 and minimal prednisone 2.

Conclusion

Relapses of GPA can occur in the absence of ANCA and even in the absence of B cells and ANCA. Patients appear to respond well to modest increases in immunosuppression. These findings suggest that there may be additional, non-ANCA, drivers of GPA disease activity.