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.

Novel Therapies in Takayasu Arteritis

“…A high initial dose of GC is recommended also by the very recent 2021 ACR guidelines, due to the potential organ damage and life-threatening events associated with TAK onset. However, ACR guidelines allows to consider lower doses for patients with newly active, non-severe disease (e.g., patients with constitutional symptoms and without limb ischemia) ( 17 ).…”

“…Given the high frequency of GC adverse effects and the high rate of relapse during tapering, the upfront use of immunosuppressives in addition to GC seems to be the most preferable management strategy in TAK patients. Based on these considerations, EULAR recommendations and ACR guidelines advise an initial treatment with high-dose GC in combination with a GC-sparing agent in all TAK patients rather than GC alone ( 16 , 17 ).…”

Temporal Artery Vascular Diseases

“…Glucocorticoids (GC) are the cornerstone of treatment for GCA. This treatment is remarkably effective but should be prescribed in high doses (40–80 mg/day prednisone-equivalent) at diagnosis for induction of remission and prevent ischemic complications [ 39 , 40 , 41 ]. In patients with GCA with acute visual loss or amaurosis fugax, the administration of 250 to 1000 mg intravenous methylprednisolone for up to 3 days should be considered [ 39 , 40 , 41 ].…”

“…This treatment is remarkably effective but should be prescribed in high doses (40–80 mg/day prednisone-equivalent) at diagnosis for induction of remission and prevent ischemic complications [ 39 , 40 , 41 ]. In patients with GCA with acute visual loss or amaurosis fugax, the administration of 250 to 1000 mg intravenous methylprednisolone for up to 3 days should be considered [ 39 , 40 , 41 ]. Once remission is achieved, the dose of prednisone is then gradually reduced, first rapidly to a target dose of 15–20 mg/day within 2–3 months and then more slowly to target ≤5 mg/day after 1 year and to be stopped after two years [ 39 , 40 , 41 ].…”

“…Methotrexate and tocilizumab, an anti-interleukin-6 (IL-6) receptor monoclonal antibody, are the two drugs used in patients with relapses or steroid-related side effects [ 45 , 46 ]. The efficacy of tocilizumab to induce and maintain remission of GCA and to significantly spare GC has been shown in two recent randomized controlled clinical trials [ 46 , 47 ]; so that recent American recommendations propose to use a combination of prednisone and tocilizumab as first-line therapy in new-onset GCA [ 40 ]. In patients receiving GC-sparing therapy, faster GC taper and earlier withdrawal of GC should be considered on an individual basis.…”