2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis

Abstract

Objective. To provide evidence-based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis.Methods. Clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to rate the quality of the evidence. Recommendations were developed by the Voting Panel, comprising adult and pediatric rheumatologists and patients. Each recommendation required ≥70% consensus among the Voting Panel.Results. We present 22 recommendations and 2 ungraded position statements for GCA, and 20 recommendations and 1 ungraded position statement for TAK. These recommendations and statements address clinical questions relating to the use of diagnostic testing, including imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA include support for the use of glucocorticoid-sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of nonglucocorticoid immunosuppressive agents with glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional due to the low quality of evidence available for most PICO questions.Conclusion. These recommendations provide guidance regarding the evaluation and management of patients with GCA and TAK, including diagnostic strategies, use of pharmacologic agents, and surgical interventions.Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to the recommendations within this guideline to be voluntary, with the ultimate determination regarding their application to be made by the physician in light of each patient's individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed and endorsed by the ACR are subject to periodic revision as warranted by the evolution of medical knowledge, technology, and practice. ACR recommendations are not intended to dictate payment or insurance decisions, and drug formularies or other third-party analyses that cite ACR guidelines should state this. These recommendations cannot adequately convey all uncertainties and nuances of patient care.

Need and value of targeted immunosuppressive therapy in giant cell arteritis

“…The use of tocilizumab in GCA is currently recommended by clinical guidelines especially in patients at high risk of GC toxicity or relapsing patients 6 31 but more recently also in new-onset GCA. 32 Less clear is what is the optimal duration of tocilizumab treatment in GCA. The available follow-up data 25 33 show that approximately half of the patients in clinical remission after 1 year of treatment maintain long-term treatment-free remission.…”

Utilization of the Revised American College of Rheumatology (rACR) Scoring to Avoid Unnecessary Temporal Artery Biopsies—A Case Series

“…This scoring system utilizes ultrasound imaging as an adjunct to compile the total score. While ultrasound imaging is being used in some centres, it is worth noting that the newly published 2021 ACR guidelines still recommend using TAB over temporal artery ultrasound [ 20 , 21 ].…”

Hidden in Plain Sight

“…Subsequent therapy involves glucocorticoid and nonglucocorticoid immunosuppressive agents such as biologic agents (tocilizumab) or oral immunosuppressants (methotrexate) as given by the 2021 American College of Rheumatology/Vasculitis Foundation guidelines for GCA and Takayasu arteritis. 10 …”