Giant Cell Arteritis (GCA) & Takayasu Arteritis (TAK) Guidelines

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. rheumatology.org/Practice-Quality/Clinical-Support/Clinical- 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 (https://www.rheumatology.org/ Practice-Quality/Clinical-Support/Clinical-Practice-Guidelines/ Vasculitis). Supplementary Appendix 1 (available on the Arthritis & Rheumatology website at http://onlinelibrary.wiley.com/ 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 (http://onlinelibrary.wiley.com/doi/10.1002/ 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.

1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, et 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum 2013;65:1–11.
2. Weyand CM, Goronzy Giant-cell arteritis and polymyalgia rheumatica [letter]. N Engl J Med 2014;371:1653.
3. Gonzalez-Gay MA, Vazquez-Rodriguez TR, Lopez-Diaz MJ, Miranda-Filloy JA, Gonzalez-Juanatey C, Martin J, et Epidemiology of giant cell arteritis and polymyalgia rheumatica [review]. Arthritis Rheum 2009;61:1454–61.
4. Koster MJ, Matteson EL, Warrington Large-vessel giant cell arteritis: diagnosis, monitoring and management [review]. Rheumatology (Oxford) 2018;57 Suppl 2:ii32–42.
5. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, et EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2009;68:318–23.
6. Salvarani C, Crowson CS, O’Fallon WM, Hunder GG, Gabriel Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum 2004;51:264–8.
7. Aiello PD, Trautmann JC, McPhee TJ, Kunselman AR, Hunder Visual prognosis in giant cell arteritis. Ophthalmology 1993;100:550–5.
8. Stone JH, Tuckwell K, Dimonaco S, Klearman M, Aringer M, Blockmans D, et al. Trial of tocilizumab in giant-cell arteritis. N Engl J Med 2017;377:317–28.
9. Hall S, Barr W, Lie JT, Stanson AW, Kazmier FJ, Hunder Takayasu arteritis: a study of 32 North American patients. Medicine (Baltimore) 1985;64:89–99.
10. Schmidt J, Kermani TA, Bacani AK, Crowson CS, Cooper LT, Matteson EL, et Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc 2013;88:822–30.
11. Sanchez-Alvarez C, Mertz LE, Thomas CS, Cochuyt JJ, Abril Demographic, clinical, and radiologic characteristics of a cohort of patients with Takayasu arteritis. Am J Med 2019;132:647–51.
12. Labarca C, Makol A, Crowson CS, Kermani TA, Matteson EL, Warrington Retrospective comparison of open versus endovascular procedures for Takayasu arteritis. J Rheumatol 2016;43:427–32.
13. Alexander PE, Li SA, Gionfriddo MR, Stoltzfus RJ, Neumann I, Brito JP, et Senior GRADE methodologists encounter challenges as part of WHO guideline development panels: an inductive content analysis. J Clin Epidemiol 2016;70:123–8.
14. Andrews J, Guyatt G, Oxman AD, Alderson P, Dahm P, Falck-Ytter Y, et GRADE guidelines: 14. Going from evidence to recommendations: the significance and presentation of recommendations. J Clin Epidemiol 2013;66:719–25.
15. Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso- Coello P, et GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008;336:924–6.
16. Alexander PE, Gionfriddo MR, Li SA, Bero L, Stoltzfus RJ, Neumann I, et A number of factors explain why WHO guideline developers make strong recommendations inconsistent with GRADE guidance. J Clin Epidemiol 2016;70:111–22.
17. Durling B, Toren A, Patel V, Gilberg S, Weis E, Jordan Incidence of discordant temporal artery biopsy in the diagnosis of giant cell arteritis. Can J Ophthalmol 2014;49:157–61.
18. Mahr A, Saba M, Kambouchner M, Polivka M, Baudrimont M, Brochériou I, et Temporal artery biopsy for diagnosing giant cell arteritis: the longer, the better? Ann Rheum Dis 2006;65:826–8.
19. Roth AM, Milsow L, Keltner The ultimate diagnoses of patients undergoing temporal artery biopsies. Arch Ophthalmol 1984;102:901–3.
20. Achkar AA, Lie JT, Hunder GG, O’Fallon WM, Gabriel How does previous corticosteroid treatment affect the biopsy findings in giant cell (temporal) arteritis? Ann Intern Med 1994;120:987–92.
21. Allison MC, Gallagher Temporal artery biopsy and corticosteroid treatment. Ann Rheum Dis 1984;43:416–7.
22. Breuer GS, Nesher R, Nesher Negative temporal artery biopsies: eventual diagnoses and features of patients with biopsy-negative giant cell arteritis compared to patients without arteritis. Clin Exp Rheumatol 2008;26:1103–6.
23. Bury D, Joseph J, Dawson Does preoperative steroid treatment affect the histology in giant cell (cranial) arteritis? J Clin Pathol 2012;65:1138–40.
24. Gonzalez-Gay MA, Garcia-Porrua C, Llorca J, Gonzalez-Louzao C, Rodriguez-Ledo Biopsy-negative giant cell arteritis: clinical spectrum and predictive factors for positive temporal artery biopsy. Semin Arthritis Rheum 2001;30:249–56.
25. Hall S, Persellin S, Lie JT, O’Brien PC, Kurland LT, Hunder The therapeutic impact of temporal artery biopsy. Lancet 1983;2:1217–20.
26. Le K, Bools LM, Lynn AB, Clancy TV, Hooks WB III, Hope The effect of temporal artery biopsy on the treatment of temporal arteritis. Am J Surg 2015;209:338–41.
27. Ray-Chaudhuri N, Kine DA, Tijani SO, Parums DV, Cartilidge N, Strong NP, et Effect of prior steroid treatment on temporal artery biopsy findings in giant cell arteritis. Br J Ophthalmol 2002;86:530–2.
28. Maleszewski JJ, Younge BR, Fritzlen JT, Hunder GG, Goronzy JJ, Warrington KJ, et Clinical and pathological evolution of giant cell arteritis: a prospective study of follow-up temporal artery biopsies in 40 treated patients. Mod Pathol 2017;30:788–96.
29. Bley TA, Uhl M, Carew J, Markl M, Schmidt D, Peter HH, et Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol 2007;28:1722–7.
30. Bowling K, Rait J, Atkinson J, Srinivas Temporal artery biopsy in the diagnosis of giant cell arteritis: does the end justify the means? Ann Med Surg (Lond) 2017;20:1–5.
31. Hussain O, McKay A, Fairburn K, Doyle P, Orr Diagnosis of giant cell arteritis: when should we biopsy the temporal artery? Br J Oral Maxillofac Surg 2016;54:327–30.
32. Luqmani R, Lee E, Singh S, Gillett M, Schmidt WA, Bradburn M, et The role of ultrasound compared to biopsy of temporal arteries in the diagnosis and treatment of giant cell arteritis (TABUL): a diagnostic accuracy and cost-effectiveness study. Health Technol Assess 2016;20:1–238.
33. Yuksel V, Guclu O, Tastekin E, Halici U, Huseyin S, Inal V, et Clinical correlation of biopsy results in patients with temporal arteritis. Rev Assoc Med Bras (1992) 2017;63:953–6.
34. Ghinoi A, Zuccoli G, Nicolini A, Pipitone N, Macchioni L, Bajocchi GL, et 1T magnetic resonance imaging in the diagnosis of giant cell arteritis: comparison with ultrasonography and physical examination of temporal arteries. Clin Exp Rheumatol 2008;26 Suppl 49:S76–80.
35. Hauenstein C, Reinhard M, Geiger J, Markl M, Hetzel A, Treszl A, et Effects of early corticosteroid treatment on magnetic resonance imaging and ultrasonography findings in giant cell arteritis. Rheumatology (Oxford) 2012;51:1999–2003.
36. Klink T, Geiger J, Both M, Ness T, Heinzelmann S, Reinhard M, et Giant cell arteritis: diagnostic accuracy of MR imaging of superficial cranial arteries in initial diagnosis-results from a multi- center trial. Radiology 2014;273:844–52.
37. Rhéaume M, Rebello R, Pagnoux C, Carette S, Clements-Baker M, Cohen-Hallaleh V, et High-resolution magnetic resonance imaging of scalp arteries for the diagnosis of giant cell arteritis: results of a prospective cohort study. Arthritis Rheumatol 2017;69:161–8.
38. Blockmans D, de Ceuninck L, Vanderschueren S, Knockaert D, Mortelmans L, Bobbaers Repetitive ¹⁸F-fluorodeoxyglucose positron emission tomography in giant cell arteritis: a prospective study of 35 patients. Arthritis Rheum 2006;55:131–7.
39. Hay B, Mariano-Goulart D, Bourdon A, Benkiran M, Vauchot F, de Verbizier D, et Diagnostic performance of ¹⁸F-FDG PET-CT for large vessel involvement assessment in patients with suspected giant cell arteritis and negative temporal artery biopsy. Ann Nucl Med 2019;33:512–20.
40. Kermani TA, Diab S, Sreih AG, Cuthbertson D, Borchin R, Carette S, et Arterial lesions in giant cell arteritis: a longitudinal study. Semin Arthritis Rheum 2019;48:707–13.
41. Nielsen BD, Gormsen LC, Hansen IT, Keller KK, Therkildsen P, Hauge Three days of high-dose glucocorticoid treatment attenuates large-vessel ¹⁸F-FDG uptake in large-vessel giant cell arteritis but with a limited impact on diagnostic accuracy. Eur J Nucl Med Mol Imaging 2018;45:1119–28.
42. Pfadenhauer K, Weinerth J, Hrdina Vertebral arteries: a target for FDG-PET imaging in giant cell arteritis? Clinical, ultrasonographic and PET study in 46 patients. Nuklearmedizin 2011;50:28–32.
43. Quinn KA, Ahlman MA, Malayeri AA, Marko J, Civelek AC, Rosenblum JS, et Comparison of magnetic resonance angiography and ¹⁸F-fluorodeoxyglucose positron emission tomography in large-vessel vasculitis. Ann Rheum Dis 2018;77:1165–71.
44. Schmidt WA, Seifert A, Gromnica-Ihle E, Krause A, Natusch Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis. Rheumatology (Oxford) 2008;47:96–101.
45. Grayson PC, Alehashemi S, Bagheri AA, Civelek AC, Cupps TR, Kaplan MJ, et ¹⁸F-fluorodeoxyglucose–positron emission tomography as an imaging biomarker in a prospective, longitudinal cohort of patients with large vessel vasculitis. Arthritis Rheumatol 2018;70:439–49.
46. Grayson PC, Tomasson G, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, et Association of vascular physical examination findings and arteriographic lesions in large vessel vasculitis. J Rheumatol 2012;39:303–9.
47. Chevalet P, Barrier JH, Pottier P, Hamidou M, Planchon B, El Kouri D, et A randomized, multicenter, controlled trial using intravenous pulses of methylprednisolone in the initial treatment of simple forms of giant cell arteritis: a one year followup study of 164 patients. J Rheumatol 2000;27:1484–91.
48. Mazlumzadeh M, Hunder GG, Easley KA, Calamia KT, Matteson EL, Griffing WL, et Treatment of giant cell arteritis using induction therapy with high-dose glucocorticoids: a double-blind, placebo- controlled, randomized prospective clinical trial. Arthritis Rheum 2006;54:3310–8.
49. Chan CC, Paine M, O’Day Steroid management in giant cell arteritis. Br J Ophthalmol 2001;85:1061–4.
50. Hayreh SS, Zimmerman B, Kardon Visual improvement with corticosteroid therapy in giant cell arteritis: report of a large study and review of literature. Acta Ophthalmol Scand 2002;80:355–67.
51. Hunder GG, Sheps SG, Allen GL, Joyce Daily and alternate- day corticosteroid regimens in treatment of giant cell arteritis: comparison in a prospective study. Ann Intern Med 1975;82:613–8.
52. Delecoeuillerie G, Joly P, de Lara AC, Paolaggi Polymyalgia rheumatica and temporal arteritis: a retrospective analysis of prognostic features and different corticosteroid regimens (11 year sur- vey of 210 patients). Ann Rheum Dis 1988;47:733–9.
53. Nesher G, Rubinow A, Sonnenblick Efficacy and adverse effects of different corticosteroid dose regimens in temporal arteritis: a retrospective study. Clin Exp Rheumatol 1997;15:303–6.
54. Villiger PM, Adler S, Kuchen S, Wermelinger F, Dan D, Fiege V, et Tocilizumab for induction and maintenance of remission in giant cell arteritis: a phase 2, randomised, double-blind, placebo-controlled trial. Lancet 2016;387:1921–7.
55. Langford CA, Cuthbertson D, Ytterberg SR, Khalidi N, Monach PA, Carette S, et A randomized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of giant cell arteritis. Arthritis Rheumatol 2017;69:837–45.
56. Mahr AD, Jover JA, Spiera RF, Hernández-García C, Fernández- Gutiérrez B, LaValley MP, et Adjunctive methotrexate for treatment of giant cell arteritis: an individual patient data meta-analysis. Arthritis Rheum 2007;56:2789–97.
57. Seror R, Baron G, Hachulla E, Debandt M, Larroche C, Puéchal X, et Adalimumab for steroid sparing in patients with giant-cell arteritis: results of a multicentre randomised controlled trial. Ann Rheum Dis 2014;73:2074–81.
58. García-Martínez A, Hernández-Rodríguez J, Grau JM, Cid Treatment with statins does not exhibit a clinically relevant corticosteroid-sparing effect in patients with giant cell arteritis. Arthritis Rheum 2004;51:674–8.
59. Narvaez J, Bernad B, Nolla JM, Valverde Statin therapy does not seem to benefit giant cell arteritis. Semin Arthritis Rheum 2007;36:322–7.
60. Pugnet G, Sailler L, Fournier JP, Bourrel R, Montastruc JL, Lapeyre- Mestre Predictors of cardiovascular hospitalization in giant cell arteritis: effect of statin exposure. A French population-based study. J Rheumatol 2016;43:2162–70.
61. Berger CT, Wolbers M, Meyer P, Daikeler T, Hess High incidence of severe ischaemic complications in patients with giant cell arteritis irrespective of platelet count and size, and platelet inhibition. Rheumatology (Oxford) 2009;48:258–61.
62. Narvaez J, Bernad B, Gómez-Vaquero C, García-Gómez C, Roig- Vilaseca D, Juanola X, et Impact of antiplatelet therapy in the development of severe ischemic complications and in the outcome of patients with giant cell arteritis. Clin Exp Rheumatol 2008;26 Suppl 49:S57–62.
63. Nesher G, Berkun Y, Mates M, Baras M, Rubinow A, Sonnenblick Low-dose aspirin and prevention of cranial ischemic complications in giant cell arteritis. Arthritis Rheum 2004;50:1332–7.
64. Salvarani C, Della Bella C, Cimino L, Macchioni P, Formisano D, Bajocchi G, et Risk factors for severe cranial ischaemic events in an Italian population-based cohort of patients with giant cell arteritis. Rheumatology (Oxford) 2009;48:250–3.
65. Hoffman GS, Cid MC, Hellmann DB, Guillevin L, Stone JH, Schousboe J, et A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum 2002;46:1309–18.
66. Spiera RF, Mitnick HJ, Kupersmith M, Richmond M, Spiera H, Peterson MG, et A prospective, double-blind, randomized, placebo controlled trial of methotrexate in the treatment of giant cell arteritis (GCA). Clin Exp Rheumatol 2001;19:495–501.
67. Hoffman GS, Cid MC, Rendt-Zagar KE, Merkel PA, Weyand CM, Stone JH, et Infliximab for maintenance of glucocorticosteroid-induced remission of giant cell arteritis: a randomized trial. Ann Intern Med 2007;146:621–30.
68. Both M, Aries PM, Muller-Hulsbeck S, Jahnke T, Schäfer PJ, Gross WL, et Balloon angioplasty of arteries of the upper extremities in patients with extracranial giant-cell arteritis. Ann Rheum Dis 2006;65:1124–30.
69. Clifford AH, Arafat A, Idrees JJ, Roselli EE, Tan CD, Rodriguez R, et Outcomes among 196 patients with noninfectious proximal aortitis. Arthritis Rheumatol 2019;71:2112–20.
70. Mennander AA, Miller DV, Liang KP, Warrington KJ, Connolly HM, Schaff HV, et Surgical management of ascending aortic aneurysm due to non-infectious aortitis. Scand Cardiovasc J 2008;42:417–24.
71. Allen DB, Growth suppression by glucocorticoid therapy [review]. Endocrinol Metab Clin North Am 1996;25:699–717.
72. Mutoh T, Shirai T, Fujii H, Ishii T, Harigae Insufficient use of corticosteroids without immunosuppressants results in higher relapse rates in Takayasu arteritis. J Rheumatol 2020;47:255–63.
73. Molloy ES, Langford CA, Clark TM, Gota CE, Hoffman Anti-tumour necrosis factor therapy in patients with refractory Takayasu arteritis: long-term follow-up. Ann Rheum Dis 2008;67:1567–9.
74. Nakaoka Y, Isobe M, Takei S, Tanaka Y, Ishii T, Yokota S, et Efficacy and safety of tocilizumab in patients with refractory Takayasu arteritis: results from a randomised, double-blind, placebo-controlled, phase 3 trial in Japan (the TAKT study). Ann Rheum Dis 2018;77:348–54.
75. Mekinian A, Resche-Rigon M, Comarmond C, Soriano A, Constans J, Alric L, et Efficacy of tocilizumab in Takayasu arteritis: multicenter retrospective study of 46 patients. J Autoimmun 2018;91:55–60.
76. Langford CA, Cuthbertson D, Ytterberg SR, Khalidi N, Monach PA, Carette S, et A randomized, double-blind trial of abatacept (CTLA-4Ig) for the treatment of Takayasu arteritis. Arthritis Rheumatol 2017;69:846–53.
77. Aeschlimann FA, Eng SW, Sheikh S, Laxer RM, Hebert D, Noone D, et Childhood Takayasu arteritis: disease course and response to therapy. Arthritis Res Ther 2017;19:255.
78. Mekinian A, Comarmond C, Resche-Rigon M, Mirault T, Kahn JE, Lambert M, et Efficacy of biological-targeted treatments in Takayasu arteritis: multicenter, retrospective study of 49 patients. Circulation 2015;132:1693–700.
79. Schmidt J, Kermani TA, Bacani AK, Crowson CS, Matteson EL, Warrington Tumor necrosis factor inhibitors in patients with Takayasu arteritis: experience from a referral center with long-term followup. Arthritis Care Res (Hoboken) 2012;64:1079–83.
80. Comarmond C, Biard L, Lambert M, Mekinian A, Ferfar Y, Kahn JE, et Long-term outcomes and prognostic factors of complications in Takayasu arteritis: a multicenter study of 318 patients. Circulation 2017;136:1114–22.
81. Gulcu A, Gezer NS, Akar S, Akkoc N, Onen F, Goktay AY. Long-term follow-up of endovascular repair in the management of arterial stenosis caused by Takayasu’s Ann Vasc Surg 2017;42:93–100.
82. De Souza AW, Machado NP, Pereira VM, Arraes AE, Neto ET, Maria HA, et Antiplatelet therapy for the prevention of arterial ischemic events in Takayasu arteritis. Circ J 2010;74:1236–41.
83. Wang X, Dang A, Lv N, Liu Q, Chen High-sensitivity C-reactive protein predicts adverse cardiovascular events in patients with Takayasu arteritis with coronary artery involvement. Clin Rheumatol 2016;35:679–84.
84. Liu YQ, Ling J, Wang Intravenous digital subtraction angiography in patients with aorto-arteritis (Takayasu’s). Cardiovasc Intervent Radiol 1990;13:83–7.
85. Lee KH, Cho A, Choi YJ, Lee SW, Ha YJ, Jung SJ, et The role of ¹⁸F-fluorodeoxyglucose–positron emission tomography in the assessment of disease activity in patients with Takayasu arteritis. Arthritis Rheum 2012;64:866–75.
86. Walter MA, Melzer RA, Schindler C, Muller-Brand J, Tyndall A, Nitzsche The value of [¹⁸F]FDG-PET in the diagnosis of large- vessel vasculitis and the assessment of activity and extent of disease. Eur J Nucl Med Mol Imaging 2005;32:674–81.
87. Vinicki JP, Garcia-Vicuna R, Arredondo M, López-Bote JP, García- Vadillo JA, Castaneda S, et Sustained remission after long-term biological therapy in patients with large vessel vasculitis: an analysis of ten cases. Reumatol Clin 2017;13:210–3.
88. Ando M, Sasako Y, Okita Y, Tagusari O, Kitamura S, Matsuo Surgical considerations of occlusive lesions associated with Takayasu’s arteritis. Jpn J Thorac Cardiovasc Surg 2000;48:173–9.
89. Lee GY, Jeon P, Do YS, Sung K, Kim DI, Kim YW, et Comparison of outcomes between endovascular treatment and bypass surgery in Takayasu arteritis. Scand J Rheumatol 2014;43:153–61.
90. Zheng T, Zhu S, Ou JF, Fang WG, Qiao ZY, Qi RD, et Treatment with corticosteroid and/or immunosuppressive agents before surgery can effectively improve the surgical outcome in patients with Takayasu’s arteritis. J Invest Surg2019;32:220–7.
91. Ham SW, Weaver Ex vivo renal artery reconstruction for complex renal artery disease. J Vasc Surg 2014;60:143–50.
92. Khalilullah M, Tyagi Percutaneous transluminal angioplasty in Takayasu arteritis. Heart Vessels Suppl 1992;7:146–53.
93. Sharma S, Gupta H, Saxena A, Kothari SS, Taneja K, Guleria S, et Results of renal angioplasty in nonspecific aortoarteritis (Takayasu disease). J Vasc Interv Radiol 1998;9:429–35.
94. Sun Y, Ma L, Ma L, Kong X, Chen H, Lv P, et Cyclophosphamide could be a better choice than methotrexate as induction treatment for patients with more severe Takayasu’s arteritis. Rheumatol Int 2017;37:2019–26.
95. Chen B, Yu HX, Zhang J, Li XX, Wu XG, Yang SJ, et Endovascular revascularization for carotid artery occlusion in patients with Takayasu arteritis. Eur J Vasc Endovasc Surg 2015;49:498–505.
96. Fields CE, Bower TC, Cooper LT, Hoskin T, Noel AA, Panneton JM, et Takayasu’s arteritis: operative results and influence of disease activity. J Vasc Surg 2006;43:64–71.
97. Kim HJ, Lee CS, Kim JS, Know SU, Kim JL, Park JW, et Outcomes after endovascular treatment of symptomatic patients with Takayasu’s arteritis. Interv Neuroradiol 2011;17:252–60.
98. Kim YW, Kim DI, Park YJ, Yang SS, Lee GY, Kim DK, et Surgical bypass vs endovascular treatment for patients with supra-aortic arterial occlusive disease due to Takayasu arteritis. J Vasc Surg 2012;55:693–700.
99. Kinjo H, Kafa The results of treatment in renal artery stenosis due to Takayasu disease: comparison between surgery, angioplasty, and stenting. A monocentrique retrospective study. G Chir 2015;36:161–7.
100. Park MC, Lee SW, Park YB, Lee SK, Choi D, Shim Post- interventional immunosuppressive treatment and vascular restenosis in Takayasu’s arteritis. Rheumatology (Oxford) 2006;45:600–5.
101. Wang X, Dang A, Lv N, Cheng N, Cheng X, Yang Y, et Long- term outcomes of coronary artery bypass grafting versus per- cutaneous coronary intervention for Takayasu arteritis patients with coronary artery involvement. Semin Arthritis Rheum 2017;47:247–52.
102. Ham SW, Kumar SR, Wang BR, Rowe VL, Weaver Late out- comes of endovascular and open revascularization for nonatherosclerotic renal artery disease. Arch Surg 2010;145:832–9.
103. Dejaco C, Ramiro S, Duftner C, Besson FL, Bley TA, Blockmans D, et EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis 2018;77:636–43.
104. Best JH, Kong AM, Unizony S, Tran O, Michalska Risk of potential glucocorticoid-related adverse events in patients with giant cell arteritis: results from a USA-based electronic health records data- base. Rheumatol Ther 2019;6:599–610.
105. Hellmich B, Agueda A, Monti S, Buttgereit F, de Boysson H, Brouwer E, et 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2020;79:19–30.