Article Text

PDF
FRI0282 Experience of Biological Agents Treatment of Refractory Takayasu Arteritis in Children
  1. Y. Kostina,
  2. G. Lyskina
  1. Department of childhood diseases, I.M. Sechenov First Moscow State Medical University, Moscow, Russian Federation

Abstract

Background Takayasu arteritis (TA) is a rare form of large vessel vasculitis characterized by granulematous inflammation. There are no controlled studies of medical treatment of children with TA.

Objectives Tumor necrosis factor (TNF) and interlecinus-6 (IL-6) is the most important in the formation of granulomas. The granulomatous nature of the histopathologic lesion in TA led us to consider an evaluation of the therapeutic benefits of anti-TNF and IL-6 therapy in patients with refractory TA.

In our study five children (5 girls) aged from 10 to 17 years with widespread inflammation in the aorta and its main branches were treated with infliximab and 1 girl was treated with tocilizumab during 12 months. The mean duration of the disease was 4.2 years. All patients received oral steroids and methotrexate or cyclophosphamide. The cause for biological treatment was standart therapy tolerance and uncontrolled arterial hypertension. Patients were tested for tuberculosis by skin test and chest roentgenography. Also we excluded active systemic infections, neutropenia, thrombocytopenia and liver dysfunction.

Methods The efficacy of therapy was evaluated by inflammation markers – erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), fibrinogen, - clinical symptoms and results of doppler ultrasound.

Results The success of 3 months biological therapy was evaluated according to the normal significances of ESP, CRP and lack of active disease symptoms, after 6 months – there were reduced of vessel wall thickness, after 12 months – clinical and laboratory remission.

Conclusions In children with refractory TA infliximab and tocilizumab therapy was associated with remission and facilitating dose reduction of corticosteroids and other immunosuppressive treatment. The presented experience justifies a randomized, controlled clinical trial of anti-TNF and IL-6 treatment of TA in children.

Disclosure of Interest None declared

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.