The European Alliance of Associations for Rheumatology (EULAR) has updated its guidance for managing polymyalgia rheumatica and primary large vessel vasculitis (LVV), emphasizing early specialist assessment, definitive diagnosis, individualized glucocorticoid tapering, and selective use of glucocorticoid-sparing therapies.
The guideline was developed according to EULAR standard operating procedures by an international task force of 28 members, including patient research partners, representing 13 European countries, Canada, India, and the US. Investigators updated the 2015 guidance for polymyalgia rheumatica (PMR) and the 2018 guidance for giant cell arteritis (GCA) and Takayasu arteritis (TAK) using systematic literature reviews of evidence published through December 31, 2024. Evidence was appraised using established risk-of-bias tools before the task force reached consensus through structured voting and anonymous agreement scoring. The process produced four overarching principles, 12 recommendations, and two quality indicators.
The recommendations address specialist referral, diagnosis, glucocorticoid therapy, adjunctive immunosuppressive treatment, relapse management, monitoring, and long-term follow-up for patients with PMR, GCA, and TAK. The guideline also includes practical treatment algorithms for each disease.
The guidance advises that all patients with suspected PMR, GCA, or TAK be referred to specialists with appropriate expertise and that patients with suspected GCA be referred within 24 hours because of the risk of irreversible ischemic complications. In patients with a strong clinical suspicion of GCA, glucocorticoid therapy should begin immediately while confirmatory investigations are pending. In contrast, glucocorticoid treatment can generally be deferred until diagnosis is confirmed in patients with suspected PMR or TAK.
The task force also emphasizes objective diagnosis. For GCA and TAK, diagnosis should be based on clinical findings and confirmed by imaging or biopsy, and classification criteria should not be used for diagnosis. For PMR, diagnosis should rely on compatible clinical features while excluding alternative conditions. The guidance also discourages empirical glucocorticoid treatment as a diagnostic test before specialist evaluation whenever possible.
Guidance on glucocorticoid therapy remains broadly consistent with previous recommendations while placing greater emphasis on achieving glucocorticoid-free remission. For newly diagnosed PMR, the recommended initial prednisone-equivalent dose is 15 to 25 mg/day, tapered to 10 mg/day within one to two months, with the goal of discontinuing therapy within one year. For new-onset GCA and newly diagnosed active TAK, recommended starting doses remain 40 to 60 mg/day, tapered to 15 to 20 mg/day within two to three months with the aim of discontinuing glucocorticoid therapy within 12 to 18 months through individualized tapering.
The document also expands the role of adjunctive therapies. Tocilizumab may be considered for selected patients with newly diagnosed PMR, whereas an interleukin-6 receptor inhibitor—preferably sarilumab, with tocilizumab as an alternative—should be considered in selected patients for relapsing or refractory PMR. Methotrexate may be used as an alternative to interleukin-6 receptor inhibitors in both groups of patients. For GCA, the task force recommends considering adjunctive tocilizumab or upadacitinib, particularly in patients with relapsing or refractory disease or those at increased risk of glucocorticoid-related adverse effects, with methotrexate as an alternative. Current safety guidance for tocilizumab and upadacitinib should also be considered. For TAK, adjunctive nonbiologic disease-modifying antirheumatic drugs should be given to all patients, with glucocorticoids used for induction of remission in patients with active disease. Tocilizumab or tumor necrosis factor inhibitors can be considered for relapsing or refractory disease despite conventional disease-modifying antirheumatic drug therapy.
The guideline also introduces a new recommendation for confirming disease relapse. In PMR and GCA, relapse should be determined through clinical assessment and laboratory markers, supported by imaging when appropriate. In TAK, imaging should play a central role alongside clinical and laboratory evaluation because disease activity may not be adequately reflected by symptoms or inflammatory markers alone.
Patients with refractory PMR, GCA, or TAK should also undergo reevaluation of the diagnosis and be considered for referral to a specialist center. For GCA and TAK, elective endovascular interventions or reconstructive surgery should be performed during stable remission, whereas arterial dissection or critical vascular ischemia requires urgent referral to a vascular team.
The guidance also advises regular monitoring for disease activity and treatment-related complications in patients with PMR, GCA, and TAK. Patients with LVV should also be screened for cardiovascular comorbidities.
The investigators acknowledged that several recommendations were informed by expert consensus because high-quality comparative evidence remains limited. They identified future research priorities that include optimizing glucocorticoid tapering, determining the optimal duration of adjunctive immunosuppressive therapy, developing disease-specific biomarkers, and clarifying the role of serial imaging in monitoring disease activity and vascular damage.
“Empirical use of GC is no longer acceptable for treating suspected PMR, GCA, or TAK. Definitive diagnosis, systematic monitoring, objective identification of relapses, escalation to adjunctive immunosuppression, and aspiring to GC-free remission should be the new mantra of managing this group of diseases with a multidisciplinary approach where the patient is a central part of the decision-making process,” wrote lead author Chetan B. Mukhtyar, of Norfolk and Norwich University Hospitals NHS Foundation Trust, Norwich, United Kingdom, and colleagues.
Disclosures: The guideline was funded by EULAR. Mukhtyar reported contracted services and expenses from Takeda UK Ltd. Multiple coauthors reported consulting fees, research funding, speaker honoraria, advisory roles, or other relationships with pharmaceutical companies, whereas several reported no competing interests. Full disclosures are available with the published guideline.
Source: Annals of the Rheumatic Diseases
