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• W4387248895 abstract "SESSION TITLE: Diffuse Lung Disease: Medications; Is It the Problem or the Solution? SESSION TYPE: Original Investigations PRESENTED ON: 10/09/2023 08:30 am - 09:30 am PURPOSE: Avacopan, an oral blocker of the C5a receptor, has recently been approved as an adjuvant to standard remission induction regimen for antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) [1]. Avacopan can reduce glucocorticoid exposure and improve renal outcomes [2, 3] . During the acute setting of diffuse alveolar hemorrhage (DAH) in AAV, questions and challenges remain related to the use of avacopan. These include the immediate effect on progression of DAH, the glucocorticoid sparing effect in the early phase of the disease, and accessibility of intubated patients to the drug. To obtain data that can inform the design of trials addressing this unresolved issue, we conducted the present study. METHODS: We performed a retrospective analysis of all consecutive patients with DAH due to AAV treated with avacopan at all Mayo Clinic sites between 1/1/2021 and 3/1/2023. The Mayo Data Explorer was used to identify patients who received avacopan prescriptions linked to a diagnosis of AAV. Inclusion criteria were age over 18 years, meeting Chapel Hill and ACR/EULAR consensus definitions for AAV, DAH, and avacopan use as part of remission induction treatment. We collected demographic data, clinical documentation, Birmingham Vasculitis Activity Scores (BVAS), imaging and laboratory results as well as medication dosing. RESULTS: Ten patients meeting eligibility criteria were identified among all Mayo Clinic sites. Patients were predominantly female (N=7) with a median age at presentation of 64 years (IQR 42-71). At the time of initiation of avacopan, 6 were newly diagnosed, and 4 had pre-existing diagnoses of AAV and 60% (N=6) were PR3 positive. Median follow up time was 12 weeks (IQR 4-22) after avacopan initiation. One patient developed respiratory failure requiring high flow nasal cannula, occurring after initiation of avacopan. Most 7 (70%) started avacopan after their hospitalization, with a median overall time to initiation of 20 (IQR 7-24) days after traditional remission induction for the whole group. Common barriers to avacopan initiation were lack of inpatient availability, provider familiarity, and concerns regarding insurance. No relapses or recurrences of DAH were observed. Nine (90%) achieved remission, 4 of whom achieved complete remission (BVAS/WG=0, off prednisone). No serious adverse events attributed to avacopan were reported. CONCLUSIONS: Although follow up was limited in this small series of patients, avacopan appears safe and effective as adjuvant to remission induction in patients with AAV and DAH. Barriers to avacopan utilization remain in clinical practice, and consensus about glucocorticoid tapering is currently lacking. CLINICAL IMPLICATIONS: With limitations inherent to retrospective reviews, future prospective studies are needed to evaluate the precise role and timing of avacopan in those with DAH and DAH. REFERENCES: 1. Kimoto, Y. and T. Horiuchi, The Complement System and ANCA Associated Vasculitis in the Era of Anti-Complement Drugs. Front Immunol, 2022. 13: p. 926044. 2. Cortazar, F., Renal Recovery for Patients with ANCA-Associated Vasculitis and Low eGFR in the ADVOCATE Trial of Avacopan. 2023. 3. Jayne, D.R.W., et al., Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med, 2021. 384(7): p. 599-609. DISCLOSURES: No relevant relationships by Rodrigo Cartin-Ceba No relevant relationships by Samuel Falde No relevant relationships by Amos Lal No relevant relationships by Augustine Lee No relevant relationships by Lester Mertz Advisory relationship with ChemoCentryx Please note: 2022 Added 10/31/2022 by Ulrich Specks, source=Web Response, value=Consulting Advisory Committee Member relationship with Astra Zeneca Please note: 2021-2023 Added 12/03/2022 by Ulrich Specks, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Boehringer Ingelheim Please note: 2022 Added 12/03/2022 by Ulrich Specks, source=Web Response, value=Consulting fee Advisory Committee Member relationship with Amgen Please note: 2023 Added 03/24/2023 by Ulrich Specks, source=Web Response, value=Consulting fee" @default.
• W4387248895 created "2023-10-03" @default.
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• W4387248895 date "2023-10-01" @default.
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• W4387248895 title "TREATMENT WITH AVACOPAN FOR ANCA-ASSOCIATED VASCULITIS WITH DIFFUSE ALVEOLAR HEMORRHAGE" @default.
• W4387248895 doi "https://doi.org/10.1016/j.chest.2023.07.2010" @default.
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