Most patients are refractory to glucocorticoids (GC) or relapse when GC doses are reduced. Early in the disease course, non-specific constitutional symptoms such as fever, malaise and weight loss may occur. Takayasu arteritis (TA) is a granulomatous vasculitis of large vessels with unknown aetiopathogenesis. The objective of this study was to determine the effectiveness of imaging modalities for the management of TAK. Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis. Assessment of the pattern and extent of arterial involvement and measurement of current disease activity are essential for the management of Takayasu arteritis (TA). As a general rule, both endovascular intervention and surgical procedures should be avoided during the active phase of the disease. We also manually searched the references of the selected articles for any relevant articles that we might have missed. Among biologic agents, TNF inhibitors (anti-TNF agents), rituximab (RTX), tocilizumab and abatacept were selected as key words. As a rule, the information obtained from non-invasive imaging methods should be integrated with patient symptoms, clinical findings and acute phase reactants to adjust the dose of IS agents and the duration of treatment. Objectives: Takayasu arteritis (TAK), is a rare autoimmune rheumatic disease causing large vessel vasculitis. This may cause extremity pain, claudication, bruits, absent or diminished pulses and loss of blood pressure. Progressive aneurysm enlargement with a tendency for dissection or rupture, severe aortic regurgitation and aortic coarctation also require surgery. Pacheco RL, Latorraca COC, de Souza AWS, Pachito DV, Riera R. Int J Clin Pract. Childhood-onset Takayasu arteritis: A 15-year experience from a tertiary referral center. In 2005 the Disease Extent Index–Takayasu (DEI-Tak) was defined for the follow-up of TA by assessing only new clinical findings within the past 6 months without the requirement for imaging techniques or acute phase reactants [ 24 ]. Published by Oxford University Press on behalf of the British Society for Rheumatology. Surgical complications such as restenosis, graft occlusion and anastomotic site aneurysm may be related to the progressive inflammatory nature of TA. The CS requirement was also reduced. The rationale of the medical treatment is to suppress systemic and vascular inflammation using CS and IS agents. In patients with LVV unable to tolerate CS, biological agents have been used with variable effectiveness. Non-invasive imaging methods are essential for monitoring disease activity and response to treatment in TA. September 2019. 2010 Jul;6(7):406-15; Hellmich B, Agueda A, Monti S, et al. 2017 Nov;71(11). CYP is a very potent and effective IS agent, generally used for the treatment of systemic vasculitis in the presence of severe life and/or vital organ–threatening conditions. Gokhan Keser, Haner Direskeneli, Kenan Aksu, Management of Takayasu arteritis: a systematic review, Rheumatology, Volume 53, Issue 5, May 2014, Pages 793–801, https://doi.org/10.1093/rheumatology/ket320. This systematic review and meta-analysis summarizes the available data on the effectiveness and safety of various small molecule IS and biological therapies for the management of TAK. Takayasu Arteritis – A Systematic Review ... Takayasu arteritis is a chronic, progressive, idiopathic, large-vessel vasculitis that affects the aorta, its main branches and the pulmonary arteries. Abstract. After summarizing the available data about medical treatment options in TA, a practical approach that also reflects our personal experiences may be recommended. In the majority of the cases, disease activity improved and CS doses were discontinued or tapered. The OMERACT Vasculitis Working Group also performs a Delphi exercise for the assessment of disease activity in LVV to develop a core set of validated outcome measures [ 27 ]. Increased vessel wall thickness, vessel wall oedema and mural contrast enhancement are usually considered evidence of active disease [ 12 ]. Recently a new version of the DEI-Tak, the Indian Takayasu’s Arteritis Score (ITAS) was introduced [ 26 ]. The authors wish to thank Professor Dr Wolfgang Schmidt, Rheumatology Medical Center, Immanuel Krankenhaus, Berlin, Germany, for his assistance in writing the imaging section. TA is a difficult disease to deal with. Later, nine additional cases of TA treated with tocilizumab 8 mg/kg every 4 weeks were reported [ 90–95 ]. Treatment duration was up to 7 years. There are case reports showing good clinical response to RTX treatment in refractory TA patients [ 86 , 87 ]. Epub 2017 Sep 12. MTX, AZA, cyclophosphamide (CYP), ciclosporin A (CSA), MMF, LEF and tacrolimus, were also used as additional key words. [ 84 ] reported five new patients and reviewed the data of 79 patients previously reported in the literature. This abstract reports the main results for Takayasu arteritis (TAK). CSA may also be effective in some cases in the treatment of pyoderma gangrenosum complicating TA [ 62–64 ]. Stent grafts are better than uncovered metal stents or PTA in terms of the patency period and occurrence of restenosis in TA patients. In patients with apparent clinical and laboratory remission, arterial specimens may show histological signs of vasculitis [ 1 , 10 ]. Our most frequent combination is MTX plus LEF, which requires close observation to avoid adverse effects. In a prospective open-label study of LEF, 15 TA patients with treatment-resistant active disease were given 20 mg/day LEF with a mean follow-up of 9.1 months. Infliximab is effective for Takayasu arteritis refractory to glucocorticoid and methotrexate, Infliximab therapy in a patient with refractory ileocolic Crohn’s disease and Takayasu arteritis, Infliximab therapy in pediatric Takayasu’s arteritis: report of two cases, Refractory Takayasu arteritis successfully treated with infliximab, Treatment of Takayasu’s arteritis with tumor necrosis factor antagonists, Takayasu arteritis: anti-TNF therapy in a Brazilian setting, Refractory Takayasu’s arteritis successfully treated with the human, monoclonal anti-tumor necrosis factor antibody adalimumab, Anti-tumor necrosis factor therapy in patients with difficult to treat Takayasu arteritis, Anti-tumor necrosis factor therapy in patients with refractory Takayasu’s arteritis: long-term follow-up, Tumor necrosis factor inhibitors in patients with Takayasu arteritis: experience from a referral center with long-term follow-up, Anti TNF-α in refractory Takayasu’s arteritis: cases series and review of the literature, Long-term efficacy and improvement of health-related quality of life in patients with Takayasu’s arteritis treated with infliximab. At the time of diagnosis, we generally start conventional IS agents together with the initial CS treatment. Anti-TNF therapy resulted in improvement in 14 of 15 patients and remission was sustained in 10 patients despite discontinuation of CS therapy. 2019 Aug 2;10:1796. doi: 10.3389/fimmu.2019.01796. There are many case reports and series showing beneficial effects in both adult and paediatric patients [ 71–80 ]. RMD Open 2019;5:e001020. In 2004, data from 15 refractory TA patients from three medical centres were reported [ 81 ]. Most reports describe a concomitant diagnosis of active TB and TA. On the other hand, long-segment stenosis with extensive periarterial fibrosis or occlusion requires surgical bypass of the affected segment, which is clearly associated with superior results compared with endovascular intervention. Since TA patients are generally immunosuppressed and often obese as the result of chronic CS therapy, surgical procedures carry additional risks. 2018 Feb;17(2):175-187. doi: 10.1016/j.autrev.2017.11.021. We conducted a comprehensive review of the literature for English articles published between 1966 and 2012, using PubMed as the database. Blood pressure measurements should be made in the unaffected extremities. While 37% of patients achieved complete remission, 53.5% showed a partial response. Since there is no completed, placebo-controlled, randomized clinical trial, the level of evidence for management of TA is low, generally reflecting the results of open studies, case series and expert opinion. Since the inner layers of the vessel wall derive nutrition from the luminal blood flow, placement of a stent graft may disturb luminal blood flow, leading to a decrease in chronic inflammation and less severe fibrotic reaction on the luminal side, with a lower incidence of restenosis [ 106 ]. Earlier diagnosis, better assessment of disease activity and future clinical trials will help improve the management of TA. When the CS dose cannot be lowered and conventional IS agents remain ineffective, or when these agents can no longer be used due to adverse events, biologic agents may be tried. Acute phase responses were significantly reduced, no adverse events occurred and control angiography showed no progression. Although there is no universally accepted consensus definition, in previous studies refractory disease was accepted if disease activity increased following reduction of the CS dose or persisted despite use of at least one conventional IS agent [ 46 ]. 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