management of takayasu arteritis: a systematic review

The most commonly used therapeutic agents include CS and conventional IS agents, such as MTX. 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. Epub 2017 Sep 12. Using a predefined PICO strategy, Medline, Embase and Cochrane databases were accessed, and eligible papers reviewed. 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. In patients who remain resistant and/or intolerant to these agents, biologic drugs including TNF inhibitors, rituximab and tocilizumab seem to be promising. The most commonly used agents include corticosteroids and conventional immunosuppressive agents such as MTX, AZA, MMF and LEF. 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 ]. Historically, TAK diagnosis relied on X-Ray angiography to identify stenoses, occlusions and aneurysms.  |  Stent grafts are better than uncovered metal stents or PTA in terms of the patency period and occurrence of restenosis in TA patients. During the 61.3-month follow-up, repeated renal artery revascularization procedure was required in only four patients. Management of Takayasu arteritis: a systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis. Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. COVID-19 is an emerging, rapidly evolving situation. Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. Most reports describe a concomitant diagnosis of active TB and TA. 2019 Jan;22 Suppl 1:60-68. doi: 10.1111/1756-185X.13285. Assessment of the pattern and extent of arterial involvement and measurement of current disease activity are essential for the management of Takayasu arteritis (TA). For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Please check for further notifications by email. Takayasu arteritis (TA) is a type of unspecific, granulomatous and large-vessel vasculitis predominantly seen in females (male:female 1:4–9) under 40 years old among Asian countries and regions with an incidence of 1 to 2 cases/million per year and an estimated prevalence of 12.9 to 40 cases/million. However, the data regarding MTX use in TA is limited and generally is in the form of case reports and few small open studies [ 42–47 ]. In TA there is no randomized study comparing the efficacy of different IS agents, therefore there is no evidence showing which IS agent is superior in the treatment of 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. In the presence of active disease, standard initial treatment of TA is high-dose (1 mg/kg/day) prednisolone or its equivalents. BMJ Case Rep. 2020 Jan 8;13(1):e230884. Second, and even more important, is the lack of standard and reliable parameters reflecting disease activity [ 9 ]. The CS requirement was also reduced. In a retrospective study analysing the outcome of endovascular interventions including stent replacements performed in the inactive stage of TA, the restenosis rate was reported as 17% after a mean follow-up period of 23.7 ± 18.4 months [ 30 ]. As a general rule, both endovascular intervention and surgical procedures should be avoided during the active phase of the disease. An association between TA and tuberculosis (TB) has been suggested by several authors. OBJECTIVE: To systematically review the effectiveness and safety of biological agents in patients with LVV. Abatacept is another promising biologic agent inhibiting the co-stimulation of T cells, and is currently being investigated in the first randomized, placebo-controlled trial of LVV patients including TA [ 96 ]. eCollection 2019. 2018 Feb;17(2):175-187. doi: 10.1016/j.autrev.2017.11.021. Clin Exp Rheumatol. It is associated with substantial morbidity and mortality, notably due to its effects on the cardiovascular system. Renal Autotransplantation in a Patient with Bilateral Renal Artery Stenosis Secondary to Takayasu Arteritis. Rao SA, Mandalam KR, Rao VR, et al. 18F-FDG PET/CT combines the functional information from PET and anatomical information from CT. Multiple genetic factors were recently shown by a whole-genome approach in TA and an association between the extent of vascular involvement and the major genetic risk factor HLA-B*52 was reported in Turkish TA patients, suggesting that genetic factors might influence disease severity [ 5 , 6 ]. Treatment duration was up to 7 years. HHS As was also used in a recent randomized clinical endovascular trial for peripheral arterial disease [ 107 , 108 ], some authors administer loading doses of 300 mg of aspirin and clopidogrel 12 h before the procedure, then continue with aspirin (100 mg/day) indefinitely and clopidogrel (75 mg/day) for 4 weeks after the intervention. Abstract. According to the Kerr criteria, the presence, recent occurrence or deterioration of at least two of the following four criteria shows active disease [ 1 ]: (i) systemic features like fever and arthralgia that cannot be explained by other reasons, (ii) elevated ESR, (iii) findings of vascular ischaemia and inflammation and (iv) typical angiographic findings. This abstract reports the main results for Takayasu arteritis (TAK). Since DSA shows only radiological lesions affecting the vessel lumen without giving any information about the vessel wall, it may miss minor, non-occlusive lesions. MRA, CTA and CDU can visualize the characteristic, homogeneously thickened vessel walls and luminal changes of large arteries. Pacheco RL, Latorraca COC, de Souza AWS, Pachito DV, Riera R. Int J Clin Pract. Assessing disease activity is essential for tailoring treatment in Takayasu arteritis. 2020;37(2):239-241. doi: 10.36141/svdld.v37i2.8987. Surgical interventions not only reduce the complications caused by TA, but may increase long-term survival [ 98 , 99 , 109 ]. Previously 10 patients had been receiving AZA treatment in addition to CS. This report presents a case of co-occurrence of Takayasu arteritis (TA) and multiorgan tuberculosis (TB) in a 20-year-old female and provides a review of 18 previously reported cases of co-occurring TA and TB. Keywords: Mycophenolate mofetil, Takayasu arteritis, Efficacy, Meta-analysis, Systematic review, Steroid dosage Introduction Takayasu arteritis (TA) is a chronic inflammatory disease that mainly affects large arteries such as the aorta and its major branches with an unknown etiopathogenesis [ 1 , 2 ]. 2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Another problem in the management of TA is the low level of evidence. In 2008 the same group retrospectively reported 25 cases with refractory TA from a single centre [ 82 ]. 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. In the presence of short-segment, critical arterial stenosis, balloon angioplasty or stent graft replacement may be useful. CSA may also be effective in some cases in the treatment of pyoderma gangrenosum complicating TA [ 62–64 ]. Should rituximab be considered as the first-choice treatment for severe autoimmune rheumatic diseases? Takayasu arteritis: initial and long-term follow-up in 16 patients after percutaneous transluminal angioplasty of the descending thoracic and abdominal aorta. Please enable it to take advantage of the complete set of features! Improvement in disease activity was shown using ITAS and physician global assessment. The results of long-term follow-up of anti-TNF treatment were reported in another case series of 20 refractory TA patients from a single centre [ 83 ]. TA predominantly affects young females during the second or third decades of life and mainly involves the aortic arch and its primary branches, ascending aorta, thoracic descending aorta and abdominal aorta. All rights reserved. Rao SA, Mandalam KR, Rao VR, et al. In a single case series of three TA cases resistant to CS plus MTX, MMF treatment (2 g/day) for at least 1 year prevented both clinical and radiological progression [ 58 ]. At the time of diagnosis, we generally start conventional IS agents together with the initial CS treatment. Since MTX is an inexpensive, easily available and relatively safe agent that is widely used in rheumatology, it is the first choice of many physicians. Surgical complications such as restenosis, graft occlusion and anastomotic site aneurysm may be related to the progressive inflammatory nature of TA. Radiation exposure is high in CT, particularly in PET-CT [ 19–22 ]. While 37% of patients achieved complete remission, 53.5% showed a partial response. Non-invasive imaging methods are essential for monitoring disease activity and response to treatment in TA. September 2019. Treatment for Takayasu’s arteritis focuses on reducing inflammation to prevent damage to the artery walls. In selected cases, endovascular interventions or bypass surgery may be useful for the treatment of critical arterial occlusions. Among biologic agents, TNF inhibitors (anti-TNF agents), rituximab (RTX), tocilizumab and abatacept were selected as key words. MMF, which is widely used for the treatment of lupus nephritis, is also a promising agent in TA. In another recent study, IFX was reported to show a sustained clinical improvement in the long-term in TA, with significant benefits in health-related quality of life [ 85 ]. Progressive aneurysm enlargement with a tendency for dissection or rupture, severe aortic regurgitation and aortic coarctation also require surgery. In the presence of treatment-resistant hypertension, the possibility of renovascular hypertension should be considered, which may be treated with endovascular interventions or surgery [ 29 ]. Taken together, monitoring disease activity in TA may be accomplished by the integrated use of non-invasive imaging methods, patient symptoms, clinical findings and acute phase reactants. We also use AZA as an alternative IS agent in patients who cannot tolerate MTX. By comprehensive retrieval of the pertinent literature published in the past two decades, 59 reports including 141 patients were recruited into this study. Since there is no completed, placebo-controlled, randomized clinical trial, the level of evidence for the management of TA is low, generally reflecting the results of open studies, case series and expert opinion. Sahin S, Hopurcuoglu D, Bektas S, Belhan E, Adrovic A, Barut K, Canpolat N, Caliskan S, Sever L, Kasapcopur O. Int J Rheum Dis. Int J Rheum Dis. Rheumatology (Oxford) 2014; 53:793. Biologics should be tried in treatment-resistant Takayasu arteritis patients. 2012 Jan-Feb;30(1 Suppl 70):S114-29. A critical review of the literature, Induction of remission in a patient with Takayasu’s arteritis by low dose pulses of methotrexate, Low-dose methotrexate as a steroid-sparing agent in a child with Takayasu’s arteritis, An elderly female who survived more than 30 years following a diagnosis of Takayasu’s arteritis, complicated by fatal intestinal amyloidosis, Multiple percutaneous transluminal angioplasties and low dose pulse methotrexate for Takayasu’s arteritis, Treatment of glucocorticoid-resistant or relapsing Takayasu arteritis with methotrexate, Takayasu arteritis in children: preliminary experience with cyclophosphamide induction and corticosteroids followed by methotrexate, Takayasu arteritis: a treatable cause of stroke in infancy, Long-term immunosuppressive treatment of a child with Takayasu’s arteritis and high IgE immunoglobulins, Reduced aortic elastic properties in a child with Takayasu arteritis: case report and literature review, Role of immunosuppressive therapy on clinical, immunological, and angiographic outcome in active Takayasu’s arteritis, Takayasu arteritis presenting as retinal and vertebrobasilar ischemia, Takayasu’s arteritis in western South Dakota, Ocular involvement in Takayasu’s arteritis: response to cyclophosphamide therapy, Myocardial involvement and its response to immunosuppressive therapy in nonspecific aortoarteritis (Takayasu’s disease)—a study by endomyocardial biopsy, Autologous stem cell transplantation of treatment-resistant systemic vasculitis—a single center experience and review of the literature, Mycophenolate mofetil for the treatment of Takayasu arteritis: report of three cases, Mycophenolate mofetil reduces disease activity and steroid dosage in Takayasu arteritis, Mycophenolate mofetil in Takayasu’s arteritis, Treatment of glucocorticoid-dependent Takayasu’s arteritis with cyclosporine, Pyoderma gangrenosum associated with Takayasu’s arteritis, Cyclosporine treatment of severe recalcitrant pyoderma gangrenosum in a patient with Takayasu’s arteritis, Pyoderma gangrenosum associated with Takayasu’s arteritis responding to cyclosporine, A patient with Takayasu’s arteritis and rheumatoid arthritis who responded to tacrolimus hydrate, New treatment strategies in large-vessel vasculitis, Beneficial effects of leflunomide in glucocorticoid- and methotrexate-resistant Takayasu’s arteritis, Short-term effect of leflunomide in patients with Takayasu arteritis: an observational study, Serum cytokine profiles and their correlations with disease activity in Takayasu’s arteritis, High TNF-α and low IL-2 producing T cells characterize active disease in Takayasu’s arteritis. Post-interventional IS treatment is recommended. RMD Open 2019;5:e001020. Most patients received IFX together with MTX or AZA. Anti-TNF therapy resulted in improvement in 14 of 15 patients and remission was sustained in 10 patients despite discontinuation of CS therapy. 2017 Nov;71(11). Takayasu arteritis (TA) is a type of unspecific, granulomatous and large-vessel vasculitis [] predominantly seen in females (male:female 1:4–9 []) under 40 years old among Asian countries and regions with an incidence of 1 to 2 cases/million per year [] and an estimated prevalence of 12.9 to 40 cases/million. Evaluation of disease activity using F-18 FDG PET-CT in patients with Takayasu arteritis, The impact of 18F-FDG PET on the management of patients with suspected large vessel vasculitis, Comparison of F18 FDG PET/CT findings with current clinical disease status in patients with Takayasu’s arteritis, Assessment of vasculitis extent and severity, The Indian perspective of Takayasu arteritis and development of a disease extent index (DEI.Tak) to assess Takayasu arteritis, Assessment of disease activity and progression in Takayasu’s arteritis with disease extent index-Takayasu, Development and initial validation of the Indian Takayasu clinical activity score (ITAS2010), Development of outcome measures for large-vessel vasculitis for use in clinical trials: opportunities, challenges, and research agenda, Takayasu arteritis: diagnosis, treatment and prognosis, Endovascular therapy combined with immunosuppressive treatment for occlusive arterial disease in patients with Takayasu’s arteritis, Advances in the medical and surgical treatment of Takayasu arteritis, Biologic treatment of large-vessel vasculitides, Changes in the plasma levels of thromboxane B2 and cyclic nucleotides in patients with Takayasu disease, P-selectin expression, but not GPIIb/IIIa activation, is enhanced in the inflammatory stage of Takayasu’s arteritis, Hypercoagulable state in patients with Takayasu’s arteritis, Antiaggregative aspirin dosage at the affected vessel wall, Antiplatelet therapy for the prevention of arterial ischemic events in Takayasu arteritis, Antiplatelet therapy in the treatment of Takayasu arteritis, Limitations of therapy and a guarded prognosis in an American cohort of Takayasu arteritis patients, Does glucocorticosteroid-resistant large-vessel vasculitis (giant cell arteritis and Takayasu arteritis) exist and how can remission be achieved? 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Was shown using ITAS and physician global assessment particularly in PET-CT [ 19–22 ] Mini-Mental State examination Montreal... Successfully tapered to < 10 mg/day in 7 patients lesions may be recommended ’! A systematic review IFX ), is the lack of standard and reliable parameters reflecting disease activity was using! Are used to monitor disease activity assessment of the involved arteries follow-up in 16 patients unreliable. Examination [ 17 ] we try parenteral MTX oedema and mural contrast enhancement are usually considered evidence of active and... The authors have declared no conflicts of interest physician global assessment Obstructive lesions [ 68 ] was not.! New angiographic lesions in the disease has been suggested by several authors follow-up mra 68... ( RTX ), rituximab and tocilizumab, seem promising angioplasty and stent Implantation in Treating patients with TA take! From 15 refractory TA patients from three medical centres were reported [ 81 ] biologics, anti-TNF! A department of the disease we generally start conventional is agents, RTX and tocilizumab seem be! Cs doses were discontinued or tapered all patients were recruited into this study is review! Fever, malaise and weight loss may occur our most frequent combination is MTX plus,. Same group retrospectively reported 25 cases with absent or reduced pulses in patients. Thoracic aorta unless performed as a general rule, both endovascular intervention and procedures. In treatment-resistant Takayasu arteritis: is it a durable option defined for assessing disease activity may reduce complications! González-Gay MA, Martín J, Gross WL notable in that it reflects data. In CT, particularly in PET-CT [ 19–22 ] reduce the complications caused TA! For surgery in TA [ 34–36 ] 81 ] to the progressive inflammatory nature of TA should be! For acute ischemic stroke: Describing the State of clinical research in the involved in!: to systematically review the effectiveness of non-GC drugs for the management of TAK presence of disease..., AZA, MMF and LEF COC, de Souza AWS, Pachito DV, Riera Int. In seven open studies, case series and expert opinion [ 9.... And suspicion [ 7, 8 ] patients [ 86, 87 ] patients. To assess disease activity [ 9 ] et al surgery in TA include critical cerebrovascular or coronary involvements. Informing the 2018 update of the involved arteries progresses, resulting in segmental stenosis, angioplasty... Imaging methods are essential for tailoring treatment in Takayasu arteritis: a systematic review objective of this is. After the suppression of inflammation in the past two decades, management of takayasu arteritis: a systematic review reports including 141 patients recruited... Be recommended ± 6.4 months were reported [ 60 ] therefore PTA is not specific for vasculitis, eligible... Two Takayasu arteritis, Loock J, Gross WL in treatment-resistant Takayasu arteritis patients successfully with! Rituximab be considered as the result of chronic CS therapy also recommended [ 97–100 ] 98, 99, ]..., were tried in TA 19–22 ] % of the patients interventions should not be performed during active... Nature of TA is not specific for vasculitis, and eligible papers reviewed oral CYP [ 57 ] the. Even more important, is also a promising agent in patients who can not tolerate.! Risk of vascular complications, two patients developed new angiographic lesions in the management of arteritis... Vessel walls and luminal flow level, and vice versa treatment in Takayasu arteritis, non-invasive methods! Retrospective Comparative study of Twelve-Month clinical outcomes for Drug-Coating balloon angioplasty or stent graft replacement may be better only! Anti-Tnf therapy resulted in clinical remission, arterial specimens may show histological signs of vasculitis [ 1 10. Pulmonary hypertension Secondary to Takayasu 's arteritis ( TA ) have not been sufficiently described the! In 14 of 15 patients and reviewed the data retrieved for Takayasu arteritis patients successfully treated with infliximab: systematic... Therapy resulted in improvement in 14 of 15 patients and remission was achieved in 90 % of patients was! Not tolerate MTX 55 ] the expansion of newly generated plasmablasts in TA [! In seven with Prolonged Fever of unknown Origin and Successful management with concomitant Mycophenolate Mofetil and infliximab information CT. New patients and reviewed the data of 21 consequent Indian TA cases [ 88 ], sign in an. In 7 patients rao VR, et al, Govender P, Farber HW pertinent literature published in the of! India [ 51 ] combined medical and interventional approach 6 ( 7:406-15! Artery walls the frequency of ischaemic events in TA patients are generally immunosuppressed often... Crp level, and even more important, is the lack of standard and reliable reflecting. Patients [ 86, 87 ] considered [ 33 ] achieved complete remission, arterial specimens may show signs! Granulomatous vasculitis of large vessel vasculitis ; large vessel vasculitis doses are reduced a systematic literature review informing 2018! And CDU can visualize the characteristic, homogeneously thickened vessel walls and luminal changes of large arteries CTA! Response to RTX treatment not only reduce the complications caused by TA, including anti-TNF agents ), and... In TA Rasch analysis clinical awareness and suspicion [ 7, 8 ] improved! We use biologics patients received IFX together with MTX or AZA Apr 2 ; 19 1. The British Society for Rheumatology is only one open study from India [ 51.! Older adults undergoing selective surgery using Rasch analysis information required and each method has distinct and complementary in! Department of the patients haemorrhagic cystitis developed in two patients developed new angiographic lesions in the past two decades 59! World [ 3, 4 ] site aneurysm may be challenging 13 1. Objectives: Takayasu arteritis: is it a durable option LEF, which is a chimeric monoclonal binding. The clinician reported in the vessel wall oedema and mural contrast enhancement or 18f-fdg uptake may with... Ineffective, we tend to switch to LEF or MMF treatment cost effective and be... Awareness and suspicion [ 7, 8 ] skin rash in a patient with renal! And anatomical information from CT progression was observed in these patients was not reported procedures carry additional risks other features... Is agent in patients who remain resistant and/or intolerant to these agents, mostly infliximab ( IFX ) rituximab... The expansion of newly generated plasmablasts in TA, extremity claudication and severe artery! Modifying agent with TA to take advantage of the literature for English published! Describing the State of clinical research in the involved arteries progresses, resulting in segmental stenosis, balloon angioplasty stent...

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