275. Maintenance of Remission and Risk of Relapse in Myeloperoxidase Positive Antineutrophil Cytoplasmic Antibody-Associated Vasculitis Glomerulonephritis
Creators: name: Moura1, Marta Casal
name: Tehranian2, Shahrzad
name: Shane A. Bobart2, 3
name: Zand2, Ladan
name: 2Eddie Greene2
Publisher: name: Zenodo
Year: 2022
Subjects: Vasculitis
ANCA
Abstracts
Giant Cell Arthritis
MPA
GPA
EGPA
Takayasu
IgA vasculitis

description: <strong>Background:</strong> Optimal time of remission-maintenance therapy in patients with MPO-ANCA associated vasculitis (MPO-AAV) is not established. Defining clinical and laboratory parameters to guide safe withdrawal of maintenance therapy is required to mitigate the risk of relapse and unnecessary exposure to immunosuppression. <strong>Methods:</strong> A retrospective cohort study of all patients followed at the Mayo Clinic with MPO-AAV (MPA and GPA) and glomerulonephritis (MPO-AAV-GN). Relapse rate, correlation with MPO-ANCA status and remission-maintenance therapies were characterized. <strong>Results:</strong> We analyzed 159 patients with active MPO-ANCA-GN. A total of 66 (41.5%) patients had at least 1 relapse. Patients with MPO-ANCA who became persistently negative did not relapse (HR 0.028, [95%CI,0.003–0.272], p=0.002). The reappearance of MPO-ANCA in patients who had turned MPO-ANCA negative was associated with increased risk of relapse (HR 2.386, [95%CI,1.471–3.870], p&lt;0.0001). Immunosuppression was withdrawn in 80 (50.3%) patients; this was less likely to occur in those who received CYC for remission-induction or were persistently MPO-ANCA positive (OR 0.443, [95%CI,0.228–0.861], p=0.016; OR 0.418, [95%CI,0.213–0.820], p=0.011, respectively). Relapse incidence was not different between patients who withdrew remission-maintenance therapy versus those who maintained (40.0% vs. 43.0%, p=0.697), even in persistently MPO-ANCA positive (39.1% vs. 48.8%, p=0.508). ENT involvement (OR 6.095 [95%CI, 1.280–29.010], p=0.023) and MPO-ANCA reappearance (OR 9.248, [95%CI, 3.126–27.361], p&lt;0.0001), were independent predictive factors for relapse after treatment withdrawal. <strong>Conclusion:</strong> Our results indicate that patients who turn and remain MPO-ANCA negative during remission are at low risk for subsequent relapse even when remission maintenance therapy is withdrawn. Subsequent reappearance of MPO-ANCA is associated with an increase of the relapse risk. Serial MPO-ANCA determinations provide useful information for clinical decision-making about remission-maintenance treatment in patients with MPO-AAV-GN. <strong>Disclosures:</strong> Dr. U. Specks has received consulting fees from AstraZeneca, ChemoCentryx and research grants from AstraZeneca, GSK, Bristol Myers Squibb, Genentech. Dr. F.C. Fervenza has received research grants from Roche/Genentech <strong>Figure 1.</strong> Algorithm for the clinical use of MPO-ANCA status for remission-maintenance guidance in MPO-AAV-GN: proposal for clinical follow-up based on the MPO-ANCA status. <sup>1. </sup>The ideal interval monitoring MPO-ANCA negative with ANCA and B-cell levels is between 3 to 4 months according to the time to MPO-ANCA reappearance and the time to relapse. <sup>2. </sup>In accordance with the most recent guidelines and clinical trials, RTX is favored as the treatment of choice for remission-maintenance. .