General Information: 2021 ACR/VF Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis

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.

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Mehrdad Maz,1Sharon A. Chung,2 Andy Abril,3 Carol A. Langford,4 Mark Gorelik,5 Gordon Guyatt,6 Amy M. Archer,7 Doyt L. Conn,8Kathy A. Full,9 Peter C. Grayson,10Maria F. Ibarra,11 Lisa F. Imundo,5 Susan Kim,2 Peter A. Merkel,12Rennie L. Rhee,12Philip Seo,13 John H. Stone,14Sangeeta Sule,15Robert P. Sundel,16 Omar I. Vitobaldi,17 Ann Warner,18 Kevin Byram,19 Anisha B. Dua,7 Nedaa Husainat,20Karen E. James,21 Mohamad A. Kalot,22Yih Chang Lin,23 Jason M. Springer,1Marat Turgunbaev,24 Alexandra Villa-Forte,4 Amy S. Turner,24 and Reem A. Mustafa25

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.

The American College of Rheumatology is an independent, professional, medical and scientific society that does not guarantee, warrant, or endorse any commercial product or service.

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.

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are systemic vasculitides that primarily affect large- and medium-sized vessels (1). GCA can present with both cranial and extracranial manifestations. Cranial manifestations include headaches, scalp tenderness, vision loss, and jaw claudication. Large vessel (“extracranial”) involvement results in arterial stenosis and aneurysms, causing absent pulses and limb claudication (2). GCA is more common in individuals of Northern European descent who are older than 50 years of age. Diagnosis is based on clinical presentation, pathologic abnormalities on temporal artery biopsy, and/or evidence of large vessel involvement on vascular imaging (1–6). Glucocorticoids are the mainstay treatment for GCA, but tocilizumab has been approved by the US Food and Drug Administration for the treatment of GCA (7,8).

TAK causes granulomatous inflammation of the aorta and its branches. It is more common in younger women (9,10). Clinical manifestations include constitutional symptoms, elevated levels of inflammation markers, and arterial stenosis and/or aneurysms resulting in limb claudication and absent pulses (11). Treatment options include glucocorticoids, nonglucocorticoid immunosuppressive agents, and surgical management of vascular abnormalities (12).

As GCA and TAK share clinical manifestations, similar questions arise regarding their treatment and management. Recent studies have broadened treatment options for GCA, and vascular imaging is increasingly used for diagnosis and management. This guideline was developed to provide evidence-based recommendations for the evaluation and management of GCA and TAK.

This guideline followed the American College of Rheumatology (ACR) guideline development process (https://www. Practice-Guidelines) using the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology to rate the quality of evidence and develop recommendations (13–15). ACR policy guided the management of conflicts of interest and disclosures ( Practice-Quality/Clinical-Support/Clinical-Practice-Guidelines/ Vasculitis). Supplementary Appendix 1 (available on the Arthritis & Rheumatology website at doi/10.1002/art.41774/abstract) presents a detailed descrip- tion of the methods. Briefly, the Literature Review team undertook systematic literature reviews for predetermined questions specifying the clinical population, intervention, comparator, and outcomes (PICO). An in-person Patient Panel of 11 individuals with different types of vasculitis (3 patients with GCA or TAK) was moderated by a member of the Literature Review team (ABD). This Patient Panel reviewed the evidence report (along with a summary and interpretation by the moderator) and provided patient perspectives and preferences about their personal experiences regarding clinical and treatment aspects of their disease. The Voting Panel comprised 9 adult rheumatologists, 5 pediatric rheumatologists, and 2 patients; they reviewed the Literature Review team’s evidence summaries and, bearing in mind the Patient Panel’s deliberations, formulated and voted on recommendations. A recommendation required ≥70% consensus among the Voting Panel.

How to interpret the recommendations

A strong recommendation is typically supported by moderate- to high-quality evidence (e.g., multiple randomized controlled trials). For a strong recommendation, the recommended course of action would apply to all or almost all patients. Only a small proportion of clinicians/patients would not want to follow the recommendation. In rare instances, a strong recommendation may be based on very low– to low-certainty evidence. For example, an intervention may be strongly recommended if it is considered low-cost, without harms, and the consequence of not performing the intervention may be catastrophic. An intervention may be strongly recommended against if there is high certainty that the intervention will lead to more harm than the comparison with very low or low certainty about its benefit (16).

A conditional recommendation is generally supported by lower-quality evidence or a close balance between desirable and undesirable outcomes. For a conditional recommendation, the recommended course of action would apply to the majority of the patients, but the alternative is a reasonable consideration. Conditional recommendations always warrant a shared decision-making approach. We specify conditions under which the alternative may be considered.

In some instances, the committee found that the evidence for a particular PICO question did not support a graded recommendation or did not favor one intervention over another. However, the Voting Panel believed that the PICO question addressed a commonly encountered clinical question which has not been fully clarified and requires further investigation, and thus felt that providing guidance for this question was warranted. For these situations, we present “ungraded position statements,” which reflect general views of the Voting Panel.

In this evidence-based guideline, we explicitly used the best evidence available and present that in a transparent manner for the clinician reader/user (10). In some instances, this includes randomized trials in which the interventions under consideration are directly compared. The GRADE system rates evidence that comes exclusively from the collective experience of the Voting Panel and Patient Panel members as “very low– quality” evidence (15).

For each recommendation, details regarding the PICO questions and the GRADE evidence tables can be found in Supplementary Appendix 2 ( art.41774/abstract).

This guideline presents the ACR/Vasculitis Foundation recommendations for the use of diagnostic testing, treatment, clinical and laboratory monitoring, and surgical intervention for patients with GCA or TAK. Overarching themes of the recommendations include the preference, in the US, for temporal artery biopsy over cranial imaging studies for the diagnosis of GCA, the use of large vessel imaging for GCA and TAK for diagnosis and disease monitoring, and limiting glucocorticoid exposure in order to minimize toxicity. Almost all recommendations are conditional due to low- quality evidence, reflecting the paucity of randomized clinical trials in these diseases.

Our recommendations regarding the use of temporal artery imaging differ from those presented by the European Alliance of Associations for Rheumatology (EULAR). In its recommendations regarding the use of imaging in large vessel vasculitis, EULAR indicates that the diagnosis of GCA may be made with a positive imaging test (e.g., temporal artery ultrasound or MRI of the cranial vessels), without additional testing such as temporal artery biopsy (103). However, the imaging recommendations presented by EULAR assume adequate expertise with these modalities. In the US, there is limited experience with temporal artery ultrasound and MRI of the cranial vessels as a diagnostic replacement for temporal artery biopsy, and thus, we continue to recommend temporal artery biopsy as the diagnostic test of choice at this time. However, we hope and anticipate that as experience with imaging of the temporal arteries to detect GCA (e.g., temporal artery ultra- sound, MRI, and/or FDG-PET) increases in the US, patients will be able to benefit from these diagnostic tests. Also, in contrast to EULAR, we favor initial treatment of GCA with glucocorticoids and a glucocorticoid-sparing agent, given the well-recognized toxicity of glucocorticoids (104,105).

When reviewing the data abstracted for the PICO questions, it was clear that many critical clinical questions remain unanswered for GCA and TAK, and the lack of sufficient clinical evidence for these questions is reflected in the ungraded position statements presented in this guideline. For example, the optimal duration of therapy for any treatment and how best to monitor disease status is unknown. Few glucocorticoid-sparing agents have been identified through high-quality data. Accurate and validated indicators of disease activity have not been established or widely used for GCA or TAK. Interpretation of imaging studies in GCA and TAK can be challenging, and the clinical significance of persistent vascular wall inflammation during clinically quiescent disease is unclear.

Given these critical gaps in knowledge, we encourage additional research into the management of GCA and TAK. Studies that may greatly benefit patient care include the following:

1) translational studies contributing to the understanding of disease pathogenesis to facilitate development of more targeted therapies;
2) randomized clinical trials identifying new therapeutic options for the management of GCA and TAK;
3) randomized clinical trials comparing the effectiveness of currently used immunosuppressive therapies; and
4) longitudinal studies with biospecimen collection and routine vascular imaging to identify biomarkers of disease activity, indicators of disease prognosis, and the clinical sequelae of abnormalities identified on vascular imaging.

We are hopeful that additional investigations into GCA and TAK will enable a more tailored approach to disease management in order to improve outcomes and minimize treatment toxicities.

We thank Anne M. Ferris, MBBS, Ora Gewurz-Singer, MD, Rula Hajj-Ali, MD, Eric Matteson, MD, MPH, Robert F. Spiera, MD, Linda Wagner-Weiner, MD, MS, and Kenneth J. Warrington, MD, for serving on the Expert Panel. We thank Antoine G. Sreih, MD and Gary S. Hoffman, MD, MS, for their contributions during the early phases of this project as members of the Core Team. Dr. Hoffman’s participation ended July 2018 due to personal reasons. Dr. Sreih’s involvement ended in December 2018 when he became primarily employed by industry, which precluded his continued participation in this project. We thank Joyce Kullman (Vasculitis Foundation) for her assistance with recruitment for the Patient Panel. We thank the patients who (along with authors Kathy A. Full and Omar I. Vitobaldi) participated in the Patient Panel meeting: Jane Ascroft, Scott A. Brunton, Dedra DeMarco, Thomas Fitzpatrick, Jenn Gordon, Maria S. Mckay, Sandra Nye, Stephanie Sakson, and Ben Wilson. We thank Robin Arnold, Catherine E. Najem, MD, MSCE, and Amit Aakash Shah, MD, MPH, for their assistance with the literature review. We thank the ACR staff, including Ms Regina Parker, for assistance in organizing the face-to- face meeting and coordinating the administrative aspects of the project, and Ms Robin Lane for assistance in manuscript preparation. We thank Ms Janet Waters for help in developing the literature search strategy and performing the initial literature search, and Ms Janet Joyce for performing the update searches.

All authors were involved in drafting the article or revising it critically for important intellectual content, and all authors approved the final version to be published. Drs. Maz, Chung, and Abril had full access to all of the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

Study conception and design. Maz, Chung, Abril, Langford, Gorelik, Guyatt, Archer, Full, Grayson, Merkel, Seo, Stone, Sule, Sundel, Vitobaldi, Turner, Mustafa.

Acquisition of data. Maz, Chung, Langford, Abril, Gorelik, Full, Imundo, Kim, Merkel, Stone, Vitobaldi, Byram, Dua, Husainat, James, Kalot, Lin, Springer, Turgunbaev, Villa-Forte, Turner, Mustafa.

Analysis and interpretation of data. Maz, Chung, Langford, Abril, Gorelik, Archer, Conn, Full, Grayson, Ibarra, Imundo, Kim, Merkel, Rhee, Seo, Stone, Vitobaldi, Warner, Byram, Dua, Husainat, Kalot, Lin, Springer, Turgunbaev, Mustafa.

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The Vasculitis Foundation would like to thank Genentech for supporting our efforts to spread the guidelines to the vasculitis community.