Predictors of medications-free and long-term remission in anti-neutrophil cytoplasmic antibody-associated vasculitis: Real-world evidence

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Parvin Babapoor
Mehrzad Hajialilo
Mehran rahimi
kamal Esalatmanesh
Dara Rahmanpour
Ali Barahimi
Alireza Khabbazi

Keywords

ANCA-associated vasculitis, remission, outcome, mortality, flare-up

Abstract

Background and aim: In this study, we report the outcomes of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in daily practice based on Connective Tissue Diseases Research Center-Vasculitis Registry (CTDRC-VR) data. Methods: Patients were included if they were 18 years or older, had a diagnosis of the groups of AAV based on 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis and microscopic polyangiitis, and were followed for a period longer than 2 years or were died. Complete clinical remission was defined as granulomatosis with polyangiitis (BVAS/GPA) of 0. Sustained remission was defined as a complete clinical remission for at least six months and tapering prednisolone dose to ≤ 7.5 mg/d. Long-term remission was defined as complete clinical remission for ≥ 5 years and tapering prednisolone dose to ≤ 7.5 mg/d. Medications-free remission was defined as complete clinical remission and discontinuation of glucocorticoids, cytotoxic medications and biologics. Results: Sixty patients with AAV were enrolled in this study. Sustained and long-term remission were developed in 91.7 and 72.1 percent of patients, respectively. Relapse was developed in 27 (45%) patients. Medications-free remission was developed in 23 (33.3%) patients. Vasculitis induced damage was developed in 40 (66.7%) patients. Patients with damage had significantly lower age and higher BVAS at the baseline. Upper airway and renal involvement, and non-adherence in patients with damage was significantly more common. Conclusions: Induction therapy leads to long-term and medications-free remission in 72% and 38% of patients with AAV, respectively.

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References

1. Yates M, Watts R. ANCA-associated vasculitis. Clin Med (Lond). 2017 Feb;17(1):60-64. doi: 10.7861/clinmedicine.
2. Samman KN, Ross C, Pagnoux C, Makhzoum JP. Update in the Management of ANCA-Associated Vasculitis: Recent Developments and Future Perspectives. Int J Rheumatol. 2021;2021:5534851. Epub 20210408. doi: 10.1155/2021/5534851.
3. Flossmann O, Berden A, de Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70(3):488-94. Epub 20101124. doi: 10.1136/ard.2010.137778.
4. Robson J, Doll H, Suppiah R, et al. Damage in the anca-associated vasculitides: long-term data from the European vasculitis study group (EUVAS) therapeutic trials. Ann Rheum Dis. 2015;74(1):177-84. Epub 20131115. doi: 10.1136/annrheumdis-2013-203927.
5. Misra DP, Agarwal V. Real-world evidence in rheumatic diseases: relevance and lessons learnt. Rheumatol Int. 2019;39(3):403-16. Epub 20190206. doi: 10.1007/s00296-019-04248-1.
6. Robson JC, Grayson PC, Ponte C, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis. 2022;81(3):315-20. Epub 20220202. doi: 10.1136/annrheumdis-2021-221795.
7. Grayson PC, Ponte C, Suppiah R, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis with Polyangiitis. Ann Rheum Dis. 2022;81(3):309-14. Epub 20220202. doi: 10.1136/annrheumdis-2021-221794.
8. Suppiah R, Robson JC, Grayson PC, et al. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for microscopic polyangiitis. Ann Rheum Dis. 2022;81(3):321-6. Epub 20220202. doi: 10.1136/annrheumdis-2021-221796.
9. Stone JH, Hoffman GS, Merkel PA, et al. A disease-specific activity index for Wegener's granulomatosis: modification of the Birmingham Vasculitis Activity Score. International Network for the Study of the Systemic Vasculitides (INSSYS). Arthritis Rheum. 2001;44(4):912-20. doi: 10.1002/1529-0131(200104)44:4<912::Aid-anr148>3.0.Co;2-5.
10. Unizony S, Villarreal M, Miloslavsky EM, et al. Clinical outcomes of treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis based on ANCA type. Ann Rheum Dis. 2016;75(6):1166-9. Epub 20151130. doi: 10.1136/annrheumdis-2015-208073.
11. Steiman AJ, Urowitz MB, Ibañez D, Papneja A, Gladman DD. Prolonged clinical remission in patients with systemic lupus erythematosus. J Rheumatol. 2014;41(9):1808-16. Epub 20140801. doi: 10.3899/jrheum.131137.
12. Malek Mahdavi A, Khabbazi A, Hajialilo M. Long-term outcome and predictors of remission in Behçet's disease in daily practice. Mod Rheumatol. 2021;31(6):1148-57. Epub 20210225. doi: 10.1080/14397595.2021.1886623.
13. Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the Vasculitis Damage Index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40(2):371-80. doi: 10.1002/art.1780400222.
14. Osterberg L, Blaschke T. Adherence to medication. N Engl J Med. 2005;353(5):487-97. doi: 10.1056/NEJMra050100.
15. Ford I, Norrie J. Pragmatic Trials. N Engl J Med. 2016;375(5):454-63. doi: 10.1056/NEJMra1510059.
16. Pagnoux C, Carette S, Khalidi NA, et al. Comparability of patients with ANCA-associated vasculitis enrolled in clinical trials or in observational cohorts. Clin Exp Rheumatol. 2015;33(2 Suppl 89):S-77-83. Epub 20150526.
17. Pérez N, Gargiulo M, Suarez L, Khoury M, Gómez G. Clinical characteristics and prognostic factors in an Argentinian cohort with ANCA-associated vasculitis. Medicina (B Aires). 2021;81(2):198-207. PubMed PMID: 33906138.
18. Rhee RL, Hogan SL, Poulton CJ, et al. Trends in Long-Term Outcomes Among Patients With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis With Renal Disease. Arthritis Rheumatol. 2016;68(7):1711-20. doi: 10.1002/art.39614.
19. Shi J, Shen Q, Chen XM, Du XG. Clinical characteristics and outcomes in microscopic polyangiitis patients with renal involvement: a study of 124 Chinese patients. BMC Nephrol. 2019;20(1):339. Epub 20190902. doi: 10.1186/s12882-019-1535-3.
20. Sada KE, Yamamura M, Harigai M, et al. Different responses to treatment across classified diseases and severities in Japanese patients with microscopic polyangiitis and granulomatosis with polyangiitis: a nationwide prospective inception cohort study. Arthritis Res Ther. 2015;17:305. Epub 20151102. doi: 10.1186/s13075-015-0815-y.
21. Toraman A, Soysal Gündüz Ö. Predictors of renal and patient outcomes in anti-neutrophil cytoplasmic antibody-associated vasculitis: Our single-center, tertiary care experience. Arch Rheumatol. 2021;36(3):445-57. Epub 20210511. doi: 10.46497/ArchRheumatol.2021.8687.

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