medical and/or surgical therapy. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Takayasu arteritis".) INFORMATION FOR PATIENTS UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." th th The Basics patient education pieces are written in plain language, at the 5 to 6 grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more th th sophisticated, and more detailed. These articles are written at the 10 to 12 grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon. Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient info" and the keyword(s) of interest.) Beyond the Basics topics (see "Patient education: Vasculitis (Beyond the Basics)") SUMMARY AND RECOMMENDATIONS The mainstay of therapy for Takayasu arteritis (TAK) is systemic glucocorticoids. However, given the chronic, relapsing nature of the disease and the imperative to avoid glucocorticoid-related toxicities, patients are often prescribed a nonglucocorticoid immunosuppressive agent in an attempt to provide both a "steroid-sparing" benefit and longer-term disease control. No specific agent has been well-proven to be effective, and it https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 12/21 7/7/23, 11:21 AM Treatment of Takayasu arteritis - UpToDate is common that patients are prescribed a series of medications, sometimes in combination. Endovascular interventions or other surgical procedures may be necessary once irreversible arterial stenosis leading to critical ischemia has occurred or large aneurysms develop. (See 'Overall approach' above.) For most patients with active TAK, we suggest initial treatment with high-dose glucocorticoids in combination with a glucocorticoid-sparing agent rather than glucocorticoids alone (Grade 2C). The choice of an additional agent in combination with glucocorticoids depends on several factors including considerations regarding comorbidities, a patient's plans for conceiving a child, cost of treatments, and availability of specific agents. (See 'Choice of glucocorticoid-sparing agent' above.) For most patients, we add either methotrexate (20 to 25 mg once weekly) or azathioprine (2 mg/kg daily) to therapy with glucocorticoids, to allow use of a lower dose of glucocorticoids while achieving or maintaining disease control. While these medications are suggested because of overall greater clinical experience, ease of use, and familiarity, reasonable alternatives include mycophenolate and leflunomide. There are no data available to clearly favor one nonbiologic disease-modifying antirheumatic drug (DMARD) over another. (See 'Nonbiologic DMARDs' above.) In selected cases, it would be reasonable to add a tumor necrosis factor (TNF) inhibitor to initial therapy with glucocorticoids. This approach depends on patient-specific preferences and circumstances, access to biologic agents, and severity of disease at presentation. (See 'Biologic DMARDs' above.) For patients with progressive disease despite initial therapy, we often combine an oral nonbiologic DMARD with a biologic DMARD, in addition to treatment as needed with glucocorticoids. The combination depends on both the perceived benefit, if any, of initial therapy and the patient's individual situation. (See 'Combination therapy' above.) Monitoring disease activity and response to therapy may be challenging for clinicians, given the absence of specific laboratory tests or validated assessment criteria for disease activity. Factors that should be taken into account when assessing disease activity include patient symptoms, physical findings, acute phase reactants, and findings on imaging. (See 'Monitoring disease activity and routine follow-up' above.) We suggest obtaining a magnetic resonance (MR) or computed tomography (CT) angiography at the time of diagnosis and then repeating the test at least annually and more often if new signs or symptoms of active disease or arterial stenosis occur. We prefer https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 13/21 7/7/23, 11:21 AM Treatment of Takayasu arteritis - UpToDate to use serial MR angiography over serial CT angiography whenever possible to avoid the additive exposure to radiation and iodinated contrast dye. (See 'Imaging' above.) Vascular intervention may be necessary for the treatment of stenosed or occluded arteries leading to organ ischemia or hypertension and for the management of aneurysmal disease. Progressive aneurysmal dilation at risk for dissection or rupture, severe aortic regurgitation (AR), and aortic coarctation also require surgery. Revascularization procedures, however, should be avoided during the active phase of the disease. (See 'Surgical management of vascular complications' above.) TAK is a chronic disease characterized by a fluctuating course, with apparent exacerbations and reductions (or remissions) in the intensity of the inflammatory processes. Only approximately one-fifth of patients have a monophasic and self-limited course, while the majority of patients show a progressive or relapsing and remitting arteritis and require long-term immunosuppressive therapies. (See 'Prognosis' above.) ACKNOWLEDGMENT The UpToDate editorial staff acknowledges Gene G Hunder, MD, who contributed to an earlier version of this topic review. Use of UpToDate is subject to the Terms of Use. REFERENCES 1. Maz M, Chung SA, Abril A, et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis. Arthritis Rheumatol 2021; 73:1349. 2. 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Topic 8219 Version 24.0 https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 18/21 7/7/23, 11:21 AM Treatment of Takayasu arteritis - UpToDate GRAPHICS Fusiform aneurysm and wall thickening in a patient with Takayasu arteritis Enlargement of the descending thoracic aorta and thickening of the vessel wall (arrows) is apparent in this contrast-enhanced CT scan. These findings suggest an active inflammatory process. CT: computed tomography. Reproduced with permission from: Sueyoshi E, Sakamoto I, Hayashi K. Aortic aneurysms in patients with Takayasu's arteritis: CT evaluation. Am J Roentgenology 2000; 175:1727. Copyright 2000 American Roentgen Ray Society. Graphic 62264 Version 6.0 https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 19/21 7/7/23, 11:21 AM Treatment of Takayasu arteritis - UpToDate Fusiform aneurysm Enlargement of the contrast-filled descending thoracic aneurysm has occurred despite treatment with glucocorticoids. Reproduced with permission from: Sueyoshi E, Sakamoto I, Hayashi K. Aortic aneurysms in patients with Takayasu's arteritis: CT evaluation. Am J Roentgenology 2000; 175:1727. Copyright 2000 American Roentgen Ray Society. Graphic 70960 Version 5.0 https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 20/21 7/7/23, 11:21 AM Treatment of Takayasu arteritis - UpToDate Contributor Disclosures Peter A Merkel, MD, MPH Equity Ownership/Stock Options: Kyverna [Systemic lupus erythematosus]. Grant/Research/Clinical Trial Support: AbbVie [Vasculitis]; AstraZeneca [Vasculitis]; Boehringer Ingelheim [Scleroderma]; Bristol-Myers Squibb [Vasculitis]; ChemoCentryx [Vasculitis]; Electra [Vasculitis]; Genentech/Roche [Vasculitis]; GlaxoSmithKline [Vasculitis]; InflaRx [Vasculitis]; Sanofi [Vasculitis]; Takeda [Vasculitis]. Consultant/Advisory Boards: AbbVie [Vasculitis]; AstraZeneca [Vasculitis]; Boehringer Ingelheim [Scleroderma]; Bristol-Myers Squibb [Vasculitis]; ChemoCentryx [Vasculitis]; CSL Behring [Scleroderma, vasculitis]; Dynacure [Vasculitis]; EMDSerono [Vasculitis]; GlaxoSmithKline [Vasculitis]; InflaRx [Vasculitis]; Janssen [Vasculitis]; Kyverna [Scleroderma, vasculitis]; MiroBio [Vasculitis]; Neutrolis [Vasculitis]; Novartis [Vasculitis]; NS Pharma [Vasculitis]; Otsuka [Vasculitis]; Q32 [Vasculitis]; Regeneron [Vasculitis]; Sparrow [Vasculitis]; Takeda [Vasculitis]. All of the relevant financial relationships listed have been mitigated. Kenneth J Warrington, MD Grant/Research/Clinical Trial Support: Eli Lilly [Giant cell arteritis]; GSK [Giant cell arteritis]; Kiniksa [Giant cell arteritis]. Other Financial Interest: Chemocentryx [Honoraria ANCA-associated vasculitis]. All of the relevant financial relationships listed have been mitigated. Philip Seo, MD, MHS No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/treatment-of-takayasu-arteritis/print 21/21