https://doi.org/10.4081/reumatismo.2021.1470
The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis
Submitted: 20 November 2021
Accepted: 24 January 2022
Published: 7 February 2022
Accepted: 24 January 2022
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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Objective: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations. Methods: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients’ representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations. Results: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids. Conclusions: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.
Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum. 1990; 33: 1135-6.
DOI: https://doi.org/10.1002/art.1780330812
Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013; 65: 1-11.
DOI: https://doi.org/10.1007/s10157-013-0869-6
Kermani TA, Warrington KJ, Crowson CS, et al. Large-vessel involvement in giant cell arteritis: a population-based cohort study of the incidence-trends and prognosis. Ann Rheum Dis. 2013; 72: 1989-94.
DOI: https://doi.org/10.1136/annrheumdis-2012-202408
Li KJ, Semenov D, Turk M, Pope J. A meta-analysis of the epidemiology of giant cell arteritis across time and space. Arthritis Res Ther. 2021; 23: 82.
DOI: https://doi.org/10.1186/s13075-021-02450-w
Mohammad AJ, Nilsson JÅ, Jacobsson LTH, et al. Incidence and mortality rates of biopsy-proven giant cell arteritis in southern Sweden. Ann Rheum Dis. 2015; 74: 993-7.
DOI: https://doi.org/10.1136/annrheumdis-2013-204652
Petri H, Nevitt A, Sarsour K, Napalkov P, Collinson N. Incidence of giant cell arteritis and characteristics of patients: data-driven analysis of comorbidities. Arthritis Care Res (Hoboken). 2015; 67: 390-5.
DOI: https://doi.org/10.1002/acr.22429
Catanoso M, Macchioni P, Boiardi L, et al. Incidence, Prevalence, and Survival of Biopsy-Proven Giant Cell Arteritis in Northern Italy During a 26-Year Period. Arthritis Care Res (Hoboken). 2017; 69: 430-8.
DOI: https://doi.org/10.1002/acr.22942
Koide K. Takayasu arteritis in Japan. Heart Vessels Suppl. 1992; 7: 48-54.
DOI: https://doi.org/10.1007/BF01744544
Mohammad AJ, Mandl T. Takayasu arteritis in Southern Sweden. J Rheumatol 2015; 42: 853-8.
DOI: https://doi.org/10.3899/jrheum.140843
Watanabe Y, Miyata T, Tanemoto K. Current clinical features of new patients with takayasu arteritis observed from cross-country research in Japan: Age and sex specificity. Circulation. 2015; 132: 1701-9.
DOI: https://doi.org/10.1161/CIRCULATIONAHA.114.012547
Hellmich B, Agueda A, Monti S, et al. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis. 2020; 79: 19-130.
DOI: https://doi.org/10.1136/annrheumdis-2019-215672
MacKie SL, Dejaco C, Appenzeller S, et al. British Society for Rheumatology guideline on diagnosis and treatment of giant cell arteritis. Rheumatol (United Kingdom). 2020; 59: E1-23.
DOI: https://doi.org/10.1093/rheumatology/keaa475
Bienvenu B, Ly KH, Lambert M, et al. Management of giant cell arteritis: Recommendations of the French Study Group for Large Vessel Vasculitis (GEFA). Rev Med Interne. 2016; 37: 154-65.
DOI: https://doi.org/10.1016/j.revmed.2015.12.015
Turesson C, Börjesson O, Larsson K, et al. Swedish Society of Rheumatology 2018 guidelines for investigation, treatment, and follow-up of giant cell arteritis. Scand J Rheumatol. 2019; 48: 259-65.
DOI: https://doi.org/10.1080/03009742.2019.1571223
Dejaco C, Ramiro S, Duftner C, et al. EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice. Ann Rheum Dis. 2018; 77: 636-43.
DOI: https://doi.org/10.1136/annrheumdis-2017-212649
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 (Hoboken, NJ). 2021; 73: 1349-65.
DOI: https://doi.org/10.1002/art.41774
Pipitone N, Olivieri I, Salvarani C. Italian Society of Rheumatology. Recommendations of the Italian Society of Rheumatology for the treatment of the primary large-vessel vasculitis with biological agents. Clin Exp Rheumatol. 2012; 30: S139-61.
Schünemann HJ, Wiercioch W, Brozek J, et al. GRADE Evidence to Decision (EtD) frameworks for adoption, adaptation, and de novo development of trustworthy recommendations: GRADE-ADOLOPMENT. J Clin Epidemiol. 2017; 81: 101-10.
DOI: https://doi.org/10.1016/j.jclinepi.2016.09.009
ISS-SNLG. Available from: https://snlg.iss.it/wp-content/uploads/2021/08/LG-342-Diagnosi-e-trattamento-vasculiti-grossi-vasi_def
Duftner C, Dejaco C, Sepriano A, et al. Imaging in diagnosis, outcome prediction and monitoring of large vessel vasculitis: A systematic literature review and meta-Analysis informing the EULAR recommendations. RMD Open. 2018; 4.
DOI: https://doi.org/10.1136/rmdopen-2017-000612
Agueda AF, Monti S, Luqmani RA, et al. Management of Takayasu arteritis: A systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis. RMD Open. 2019; 5.
DOI: https://doi.org/10.1136/rmdopen-2019-001020
Monti S, Águeda AF, Luqmani RA, et al. Systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis: Focus on giant cell arteritis. RMD Open. 2019; 5.
DOI: https://doi.org/10.1136/rmdopen-2019-001003
Brouwers MC, Kho ME, Browman GP, et al. AGREE II: Advancing guideline development, reporting, and evaluation in health care. Prev Med (Baltim). 2010; 51: 421-4.
DOI: https://doi.org/10.1016/j.ypmed.2010.08.005
Whiting PF, Rutjes AWS, Westwood ME, et al. Quadas-2: A revised tool for the quality assessment of diagnostic accuracy studies. Ann Intern Med. 2011; 155: 529-36.
DOI: https://doi.org/10.7326/0003-4819-155-8-201110180-00009
Sterne JA, Hernán MA, Reeves BC, et al. ROBINS-I: A tool for assessing risk of bias in non-randomised studies of interventions. BMJ. 2016; 355.
Sterne JAC, Savović J, Page MJ, et al. RoB 2: A revised tool for assessing risk of bias in randomised trials. BMJ. 2019; 366.
DOI: https://doi.org/10.1136/bmj.l4898
Hayden JA, van der Windt DA, Cartwright JL, et al. Assessing bias in studies of prognostic factors. Ann Intern Med. 2013; 158: 280-6.
DOI: https://doi.org/10.7326/0003-4819-158-4-201302190-00009
Ball EL, Walsh SR, Tang TY, et al. Role of ultrasonography in the diagnosis of temporal arteritis. Br J Surg. 2010; 97: 1765-71.
DOI: https://doi.org/10.1002/bjs.7252
Ješe R, Rotar Ž, Tomšič M, Hočevar A. The role of colour doppler ultrasonography of facial and occipital arteries in patients with giant cell arteritis: A prospective study. Eur J Radiol. 2017; 95: 9-12.
DOI: https://doi.org/10.1016/j.ejrad.2017.07.007
Schmidt WA, Seifert A, Gromnica-ihle E, et al. Ultrasound of proximal upper extremity arteries to increase the diagnostic yield in large-vessel giant cell arteritis. Rheumatology. 2008; 47: 96-101.
DOI: https://doi.org/10.1093/rheumatology/kem322
Pérez López J, Solans Laqué R, Bosch Gil JA, et al. Colour-duplex ultrasonography of the temporal and ophthalmic arteries in the diagnosis and follow-up of giant cell arteritis. Clin Exp Rheumatol. 2009; 27.
Karassa FB, Matsagas MI, Schmidt WA, Ioannidis JPA. Meta-analysis: test performance of ultrasonography for giant-cell arteritis. Ann Intern Med. 2005; 142: 359-69.
DOI: https://doi.org/10.7326/0003-4819-142-5-200503010-00011
Luqmani R, Lee E, Singh S, et al. 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 (Rockv). 2016; 20: 1-270.
DOI: https://doi.org/10.3310/hta20900
Chrysidis S, Døhn UM, Terslev L, et al. Diagnostic accuracy of vascular ultrasound in patients with suspected giant cell arteritis (EUREKA): a prospective, multicentre, non-interventional, cohort study. Lancet Rheumatol. 2021; 0.
DOI: https://doi.org/10.1016/S2665-9913(21)00246-0
Bley TA, Uhl M, Carew J, et al. Diagnostic value of high-resolution MR imaging in giant cell arteritis. AJNR Am J Neuroradiol. 2007; 28: 1722-7.
DOI: https://doi.org/10.3174/ajnr.A0638
Franke P, Markl M, Heinzelmann S, Vaith P, et al. Evaluation of a 32-channel versus a 12-channel head coil for high-resolution post-contrast MRI in giant cell arteritis (GCA) at 3T. Eur J Radiol. 2014; 83: 1875-80.
DOI: https://doi.org/10.1016/j.ejrad.2014.06.022
Klink T, Geiger J, Both M, et al. Giant cell arteritis: diagnostic accuracy of MR imaging of superficial cranial arteries in initial diagnosis-results from a multicenter trial. Radiology. 2014; 273: 844-52.
DOI: https://doi.org/10.1148/radiol.14140056
Poillon G, Collin A, Benhamou Y, et al. Increased diagnostic accuracy of giant cell arteritis using three-dimensional fat-saturated contrast-enhanced vessel-wall magnetic resonance imaging at 3 T. Eur Radiol. 2020; 30: 1866-75.
DOI: https://doi.org/10.1007/s00330-019-06536-7
Rhéaume M, Rebello R, Pagnoux C, et al. 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.
DOI: https://doi.org/10.1002/art.39824
Nielsen BD, Gormsen LC, Hansen IT, et al. 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.
DOI: https://doi.org/10.1007/s00259-018-4021-4
Nielsen BD, Hansen IT, Keller KK, et al. Diagnostic accuracy of ultrasound for detecting large-vessel giant cell arteritis using FDG PET/CT as the reference. Rheumatology (Oxford). 2020; 59: 2062-73.
DOI: https://doi.org/10.1093/rheumatology/kez568
Grayson PC, Alehashemi S, Bagheri AA, et al. 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.
DOI: https://doi.org/10.1002/art.40379
Schramm N, Ingenhoff J, Dechant C, et al. Diagnostic accuracy of positron emission tomography for assessment of disease activity in large vessel vasculitis. Int J Rheum Dis. 2019; 22: 1371-7.
DOI: https://doi.org/10.1111/1756-185X.13440
MacChioni P, Boiardi L, Muratore F, et al. Survival predictors in biopsy-proven giant cell arteritis: A northern Italian population-based study. Rheumatol (United Kingdom). 2019; 58: 609-16.
DOI: https://doi.org/10.1093/rheumatology/key325
Dagna L, Salvo F, Tiraboschi M, et al. Pentraxin-3 as a marker of disease activity in Takayasu arteritis. Ann Intern Med. 2011; 155: 425-33.
DOI: https://doi.org/10.7326/0003-4819-155-7-201110040-00005
Park MC, Lee SW, Park YB, Lee SK. Serum cytokine profiles and their correlations with disease activity in Takayasu’s arteritis. Rheumatology. 2006; 45: 545-8.
DOI: https://doi.org/10.1093/rheumatology/kei266
Li ZQ, Zheng ZH, Ding J, et al. Contrast-enhanced ultrasonography for monitoring arterial inflammation in takayasu arteritis. J Rheumatol. 2019; 46: 616-22.
DOI: https://doi.org/10.3899/jrheum.180701
Raine C, Stapleton PP, Merinopoulos D, et al. A 26-week feasibility study comparing the efficacy and safety of modified-release prednisone with immediate-release prednisolone in newly diagnosed cases of giant cell arteritis. Int J Rheum Dis. 2018; 21: 285-91.
DOI: https://doi.org/10.1111/1756-185X.13149
Mazlumzadeh M, Hunder GG, Easley KA, et al. 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.
DOI: https://doi.org/10.1002/art.22163
Cacoub P, Chemlal K, Khalifa P, et al. Deflazacort versus prednisone in patients with giant cell arteritis: Effects on bone mass loss. J Rheumatol. 2001; 28: 2474-9.
Chevalet P, Barrier JH, Pottier P, et al. 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.
Patil P, Williams M, Maw WW, et al. Fast track pathway reduces sight loss in giant cell arteritis: results of a longitudinal observational cohort study. Clin Exp Rheumatol. 2015; 33: 2-5.
DOI: https://doi.org/10.1093/rheumatology/keu191
Diamantopoulos AP, Haugeberg G, Lindland A, Myklebust G. The fast-track ultrasound clinic for early diagnosis of giant cell arteritis significantly reduces permanent visual impairment: Towards a more effective strategy to improve clinical outcome in giant cell arteritis? Rheumatol (United Kingdom). 2016; 55: 66-70.
DOI: https://doi.org/10.1093/rheumatology/kev289
Touma Z, Nawwar R, Hadi U, et al. The use of TNF-α blockers in Cogan’s syndrome. Rheumatol Int. 2007; 27: 995-6.
DOI: https://doi.org/10.1007/s00296-007-0373-y
Langford CA, Cuthbertson D, Ytterberg SR, et al. A Randomized, Double-Blind Trial of Abatacept (CTLA-4Ig) for the Treatment of Takayasu Arteritis. Arthritis Rheumatol. 2017; 69: 846-53.
DOI: https://doi.org/10.1002/art.40037
Kyle V, Hazleman BL. Treatment of polymyalgia rheumatica and giant cell arteritis. I. Steroid regimens in the first two months. Ann Rheum Dis. 1989; 48: 658-61.
DOI: https://doi.org/10.1136/ard.48.8.658
Kermani TA, Warrington KJ, Cuthbertson D, et al. Disease relapses among patients with giant cell arteritis: A prospective, longitudinal cohort study. J Rheumatol. 2015; 42: 1213-7.
DOI: https://doi.org/10.3899/jrheum.141347
Hoffman GS, Cid MC, Hellmann DB, et al. A multicenter, randomized, double-blind, placebo-controlled trial of adjuvant methotrexate treatment for giant cell arteritis. Arthritis Rheum. 2002; 46: 1309-18.
DOI: https://doi.org/10.1002/art.10262
Spiera RF, Mitnick HJ, Kupersmith M, et al. trial of methotrexate in the treatment of giant cell arteritis ( GCA ). Trial. 2001; 495-501.
Jover JA, Hernández-García C, Morado IC, et al. Combined treatment of giant-cell arteritis with methotrexate and prednisone: A randomized, double-blind, placebo-controlled trial. Ann Intern Med. 2001; 134: 106-14.
DOI: https://doi.org/10.7326/0003-4819-134-2-200101160-00010
Mahr AD, Jover JA, Spiera RF, et al. Adjunctive methotrexate for treatment of giant cell arteritis: An individual patient data meta-analysis. Arthritis Rheum. 2007; 56: 2789-97.
DOI: https://doi.org/10.1002/art.22754
Stone JH, Tuckwell K, Dimonaco S, et al. Trial of Tocilizumab in Giant-Cell Arteritis. N Engl J Med. 2017; 377: 317-28.
DOI: https://doi.org/10.1056/NEJMoa1613849
Villiger PM, Adler S, Kuchen S, et al. 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.
DOI: https://doi.org/10.1016/S0140-6736(16)00560-2
Matza MA, Fernandes AD, Stone JH, Unizony SH. Ustekinumab for the Treatment of Giant Cell Arteritis. Arthritis Care Res (Hoboken). Published Online First: 5 April 2020.
Quartuccio L, Isola M, Bruno D, et al. Treatment strategy introducing immunosuppressive drugs with glucocorticoids ab initio or very early in giant cell arteritis: A multicenter retrospective controlled study. J Transl Autoimmun. 2020; 3.
DOI: https://doi.org/10.1016/j.jtauto.2020.100072
Nakaoka Y, Isobe M, Takei S, et al. 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.
DOI: https://doi.org/10.1136/annrheumdis-2017-211878
Manara M, Ughi N, Ariani A, et al. Providing updated guidelines for the management of rheumatic diseases in Italy with the ADAPTE methodology: A project by the Italian society for rheumatology. Reumatismo. 2019; 71: 1-4.
DOI: https://doi.org/10.4081/reumatismo.2019.1186
How to Cite
Ughi, N., Padoan, R., Crotti, C., Sciascia, S., Carrara, G., Zanetti, A., … Cimmino, M. (2022). The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis. Reumatismo, 73(4). https://doi.org/10.4081/reumatismo.2021.1470
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