EULAR recommends a combination of glucocorticoids with either rituximab or cyclophosphamide for remission induction in life-threatening or organ-threatening AAV, followed by gradual tapering of glucocorticoids.1 The introduction of biologics such as rituximab as maintenance in AAV has reduced the rate of flares,3-6 but it is not clear whether concomitant low-dose glucocorticoids during the same period provides an additional benefit without excess risk. Now, new findings shed light on how low-dose glucocorticoid use impacts the risk of relapse and chronic damage during maintenance treatment for AAV. Data come from 171 patients who received maintenance treatment at three referral centres in Greece, with a median follow-up of just over 7 years. Overall, there were 65 major flares in 48 patients (8.1/100 patient-years). Rituximab use was associated with a lower risk for major flares, while disease activity at diagnosis was associated with an increased risk. Concomitant glucocorticoid use at less than 7.5 mg per day showed no protective effect against major flares – but was associated with an increased risk for damage accumulation and hospitalisations.
Presenting the work at the EULAR 2026 Congress in London, Katerina Chavatza said "Low-dose glucocortioid added to standard maintenance did not reduce the risk of major flares, but it was associated with an increased risk of chronic damage and hospitalisation. These findings support earlier and more aggressive glucorticoid withdrawal following remission in people with AAV."
Despite therapeutic advances, patients with GCA also continue to experience substantial glucocorticoid exposure, as well as frequent relapses and variable treatment responses. A poster at the congress from Omar Dhrif and colleagues proposed a first definition of difficult-to-treat GCA, including risk factors that may predispose patients to this disease course. The group analysed 546 patients from the French Large Vessel Vasculitis Study Group that fulfilled the 2022 ACR/EULAR classification criteria for GCA,7 and who had minimum follow-up of 24 months and a full documented history of monthly glucocorticoid dose and relapse history. The proposed definition has three elements: patients who received at least one immunosuppressive agent and in whom it was not possible to withdraw after 24 months of treatment due to persisting disease activity, plus an inability to taper glucocorticoid dosage below 5 mg per day due to persisting disease activity, and at least two relapses during follow-up.
In this cohort, difficult-to-treat GCA accounted for 9.7% of the cohort and was characterised by a distinct phenotype, marked by predominant large-vessel vasculitis involvement, higher female representation, and difficulty tapering glucorticoid beyond 6 months. Difficult-to-treat patients required a maintenance glucocorticoid dose of 8.8 mg per day at Month 24 versus 2.68 in those without difficult-to-treat disease. The dosage at 6 months predicted progression to difficult-to-treat GCA in females with large-vessel vasculitis.
The definition proposed in this study captures key unmet needs in GCA, including recurrent relapses and insufficient glucocorticoid sparing. These findings underscore the clinical relevance of identifying difficult-to-treat disease as a distinct subset to guide specialised management, including early introduction of a glucocorticoid-sparing agent when targets are not met at Month 6. These findings are interesting, and the predictive model warrants external validation in independent cohorts.
Source
Chavatza K, et al. In patients with ANCA-associated vasculitides, low-dose glucocorticoids added to maintenance treatment, does not reduce the risk of major flares and is associated with an increased risk for chronic damage and hospitalization. Presented at EULAR 2026; OP0229. Ann Rheum Dis 2026; DOI: 10.1136/annrheumdis-2026-eular.B.4083.
Dhrif O, et al. Identifying Difficult-to-Treat Giant Cell Arteritis: Results from a French Large Vessel Vasculitis Study Group Cohort. Presented at EULAR 2026; POS0023. Ann Rheum Dis 2026; DOI: 10.1136/annrheumdis-2026-eular.B.900.
References
1. Hellmich B, et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis 2024;83:30–47. DOI: 10.1136/ard-2022-223764.
2. Hellmich B, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020;79:19–30. DOI: 10.1136/annrheumdis-2019-215672.
3. Guillevin L, et al. Rituximab versus Azathioprine for Maintenance in ANCA-Associated Vasculitis. New England Journal of Medicine 2014;371:1771–80. DOI: 10.1056/NEJMoa1404231.
4. Terrier B, et al. Long-term efficacy of remission - maintenance regimens for ANCA-associated vasculitides. Ann Rheum Dis 2018;77:1150–56. DOI: 10.1136/annrheumdis-2017-212768.
5. Charles P, et al. Long-Term rituximab use to maintain remission of antineutrophil cytoplasmic antibody–associated vasculitis: A randomized trial. Ann Intern Med 2020;173:179–88. DOI: 10.7326/M19-3827.
6. Smith RM, et al. Rituximab versus azathioprine for maintenance of remission for patients with ANCA-associated vasculitis and relapsing disease: An international randomised controlled trial. Ann Rheum Dis 2023;82:937–44. DOI: 10.1136/ard-2022-223559.
7. Ponte C, et al. 2022 American College of Rheumatology/EULAR Classification Criteria for Giant Cell Arteritis. Arthritis Rheumatol Hoboken NJ 2022;74:1881–9. DOI: 10.1002/art.42325.
About EULAR
EULAR is the European umbrella organisation representing scientific societies, health professional associations and organisations for people with rheumatic and musculoskeletal diseases (RMDs). EULAR aims to reduce the impact of RMDs on individuals and society, as well as improve RMD treatments, prevention, and rehabilitation. To this end, EULAR fosters excellence in rheumatology education and research, promotes the translation of research advances into daily care, and advocates for the recognition of the needs of those living with RMDs by EU institutions.
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