AB1009 THE EFFICACY OF TOCILIZUMAB ON THE TREATMENT OF TAKAYASU ARTERITIS IN CHINESE CHILDREN

Background:Takayasu arteritis (TA) is the most prevalent large-vessel vasculitis in children. Patients with TA have a high mobidity and mortality.It remains a therapeutic challenge because corticosteroids monotherapy can rarely cure TAK and the relapse rate is high during GC tapering.Objectives:The aim of this study is to investigate the efficacy and safety of tocilizumab (TCZ)in Chinese children with Takayasu arteritis(TAK).Methods:We retrospectively studied 6 TAK children treated with TCZ in our hospital from July 2017 to October 2018. The demographic and clinical data, laboratory examination results and vascular imaging data were collected.Results:Six pediatric patients with critical or refractory TAK treated with TCZ were analyzed, including 3 males and 3 females.The diagnosis age was ranging in age from 2 to 13 years(median age:7 years).Three patients were initially treated with TCZ and Mycophenolate Mofetil(MMF) as the first-line regimen without corticosteroid or with a quite rapid GC taper duration,two of which had lifte-threatening coronary arteries involved and heart failure.The other three paitients were swcithed to TCZ from conventional disease modifying anti-rheumatic drugs (DMARDs) or other biologics due to being refractory to them and recurrent relapses.Four patients were given TCZ at 4 weeks regular intervals for 10 to 22 months,while two patients withdrew TCZ because of disease deterioration and unbearable abdominal or chest pain after the second dose.After 6 months follow-up,four patients experienced significant clinical and biological improvement with angiographically progression in one patient. A corticosteroid-sparing effect is obvious. Drug-related side effects occur in 1 patients manifesting as a mild elevated liver fuction. Neither neutropenia nor infection was observed.Conclusion:Our study shows a clinical, biological, and radiological response in patients with refractory TAK treated with TCZ.References :[1]Hellmich B, Agueda A, Monti S,et al.2018 Update of the EULAR recommendations for the management of large vessel vasculitis. Ann Rheum Dis 2019;0:1–12. doi:10.1136/annrheumdis-2019-215672.[2]BravoMancheño B, Perin F, Guez Vázquez Del ReyMDMR, García Sánchez A, Alcázar Romero PP. Successful tocilizumab treatment in a child with refractory Takayasu arteritis.Pediatrics 2012;130(6):e1720-724.[3]Goel R, Danda D, Kumar S, Joseph G. Rapid control of disease activity by tocilizumab in 10 «difficult-to-treat» cases of Takayasu arteritis. Int J Rheum Dis 2013;16(6):754–61.[4]Cañas CA, Cañas F, Izquierdo JH, Echeverri A-F, Mejía M, Bonilla-Abadía F, et al. Efficacy and safety of anti-interleukin 6 receptor monoclonal antibody (tocilizumab) in Colombian patients with Takayasu arteritis. J Clin Rheumatol Pract Rep Rheum Musculoskelet Dis 2014;20(3):125–9.[5]Batu ED, Sönmez HE, Hazirolan T, Özaltin F, Bilginer Y, Özen S. Tocilizumab treatment in childhood Takayasu arteritis: case series of four patients and systematic review of the literature. Semin Arthritis Rheum 2017 Feb;46(4):529–35.Disclosure of Interests:None declared

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THU0286 MANAGEMENT OF TAKAYASU ARTERITIS: A SYSTEMATIC LITERATURE REVIEW INFORMING THE 2018 UPDATE OF THE EULAR RECOMMENDATIONS FOR THE MANAGEMENT OF LARGE VESSEL VASCULITIS

10.1136/annrheumdis-2019-eular.7702 ◽

2019 ◽

Cited By ~ 1

Author(s):

Ana Filipa Águeda ◽

Sara Monti ◽

Raashid Luqmani ◽

Frank Buttgereit ◽

Maria C. Cid ◽

...

Keyword(s):

Literature Review ◽

Systematic Literature Review ◽

Takayasu Arteritis ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Eular Recommendations

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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 ◽

10.1136/rmdopen-2019-001020 ◽

2019 ◽

Vol 5 (2) ◽

pp. e001020 ◽

Cited By ~ 12

Author(s):

Ana F Águeda ◽

Sara Monti ◽

Raashid Ahmed Luqmani ◽

Frank Buttgereit ◽

Maria Cid ◽

...

Keyword(s):

Takayasu Arteritis ◽

Meta Analysis ◽

Case Series ◽

Immunosuppressive Drugs ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Inactive Disease ◽

Controlled Clinical Trial ◽

Level Of Evidence ◽

Randomised Controlled

ObjectiveTo collect available evidence on management of large vessel vasculitis to inform the 2018 update of the EULAR management recommendations.MethodsTwo independent systematic literature reviews were performed, one on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO (population, intervention, comparator and outcome) strategy, Medline, Embase and Cochrane databases were accessed. Eligible papers were reviewed and results condensed into a summary of findings table. This paper reports the main results for Takayasu arteritis (TAK).ResultsA total of 287 articles were selected. Relevant heterogeneity precluded meta-analysis. Males appear to have more complications than females. The presence of major complications, older age, a progressive disease course and a weaker inflammatory response are associated with a more unfavourable prognosis. Evidence for details on the best disease monitoring scheme was not found. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids are widely accepted as first-line treatment. Conventional immunosuppressive drugs and tumour necrosis factor inhibitors were beneficial in case series and uncontrolled studies. Tocilizumab failed the primary endpoint (time to relapse) in a randomised controlled clinical trial; however, results still favoured tocilizumab over placebo. Vascular procedures may be required, and outcome is better when performed during inactive disease.ConclusionsEvidence to guide monitoring and treatment of patients with TAK is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.

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EULAR recommendations for the management of large vessel vasculitis

Annals of the Rheumatic Diseases ◽

10.1136/ard.2008.088351 ◽

2008 ◽

Vol 68 (3) ◽

pp. 318-323 ◽

Cited By ~ 408

Author(s):

C Mukhtyar ◽

L Guillevin ◽

M C Cid ◽

B Dasgupta ◽

K de Groot ◽

...

Keyword(s):

Large Vessel Vasculitis ◽

Large Vessel ◽

Level Of Evidence ◽

Drug Regulatory Agency ◽

Modified Delphi ◽

Eular Recommendations ◽

European League Against Rheumatism ◽

The Usa ◽

Modified Delphi Technique ◽

Management Recommendations

Objectives:To develop European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis.Methods:An expert group (10 rheumatologists, 3 nephrologists, 2 immunolgists, 2 internists representing 8 European countries and the USA, a clinical epidemiologist and a representative from a drug regulatory agency) identified 10 topics for a systematic literature search through a modified Delphi technique. In accordance with standardised EULAR operating procedures, recommendations were derived for the management of large vessel vasculitis. In the absence of evidence, recommendations were formulated on the basis of a consensus opinion.Results:Seven recommendations were made relating to the assessment, investigation and treatment of patients with large vessel vasculitis. The strength of recommendations was restricted by the low level of evidence and EULAR standardised operating procedures.Conclusions:On the basis of evidence and expert consensus, management recommendations for large vessel vasculitis have been formulated and are commended for use in everyday clinical practice.

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Reducing the Toxicity of Long-Term Glucocorticoid Treatment in Large Vessel Vasculitis

Current Rheumatology Reports ◽

10.1007/s11926-020-00961-0 ◽

2020 ◽

Vol 22 (12) ◽

Author(s):

Andriko Palmowski ◽

Frank Buttgereit

Keyword(s):

Adverse Events ◽

Takayasu Arteritis ◽

Term Treatment ◽

Large Vessel Vasculitis ◽

Large Vessel ◽

Serious Adverse Events ◽

Glucocorticoid Treatment ◽

Long Term Treatment ◽

Higher Doses

Abstract Purpose While glucocorticoids (GCs) are effective in large vessel vasculitis (LVV), they may cause serious adverse events (AEs), especially if taken for longer durations and at higher doses. Unfortunately, patients suffering from LVV often need long-term treatment with GCs; therefore, toxicity needs to be expected and countered. Recent Findings GCs remain the mainstay of therapy for both giant cell arteritis and Takayasu arteritis. In order to minimize their toxicity, the following strategies should be considered: GC tapering, administration of conventional synthetic (e.g., methotrexate) or biologic (e.g., tocilizumab) GC-sparing agents, as well as monitoring, prophylaxis, and treatment of GC-related AEs. Several drugs are currently under investigation to expand the armamentarium for the treatment of LVV. Summary GC treatment in LVV is effective but associated with toxicity. Strategies to minimize this toxicity should be applied when treating patients suffering from LVV.

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