Abstract

Behcet’s disease (BD) is a large vessel vasculitis with a wide range of clinical manifestations. Some of these manifestation may be life threatening and rapid suppression of the inflammation with effective immunosuppressive agent is crucial. There are traditional drugs with different response rates and all have efficacy on different manifestations of the disease. The most frightening manifestations of the disease are ocular, neurologic, intestinal and vascular types of involvement. Besides benign and easily treated manifestations there are also refractory cases with complicated involvement. The novel biologic agents have been used for these resistant patients and favorable response rates have been reported. In this review, we have shared our experience with biologic agents in BD and also reviewed the literature for the efficacy and safety for these novel agents for refractory patients.

Highlights

  • Behçet’s disease (BD) is a systemic large-vessel vasculitis with various clinical manifestations like recurrent oral and genital aphthous ulcerations, uveitis, vascular, neurological, articular and gastrointestinal manifestations [1] [2]

  • Uveitis is the most frequent complication of BD and besides interferon alfa, infliximab, adalimumab, anakinra, canakinumab, tocilizumab and ruti-ximab are the novel agents for resistant diseases with favorable efficacy and tolerability [5]

  • For refractory ocular disease IFN alpha 2a, infliximab and perhaps adalimumab may be effective treatment options but further trials are necessary with other novel biologic agents

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Summary

Introduction

Behçet’s disease (BD) is a systemic large-vessel vasculitis with various clinical manifestations like recurrent oral and genital aphthous ulcerations, uveitis, vascular, neurological, articular and gastrointestinal manifestations [1] [2]. Colchicine and some nonsteroidal anti-inflammatory agents are generally efficacious for muco-cutaneous and joint symptoms [3]. For patients with posterior uveitis, vasculitis, and patients with neurological and gastrointestinal involvements, aggressive immunosuppressive agents are necessary [4]. BD may present with neurologic symptoms as parenchymal or nonparenchymal neuro-Behçet disease (NBD). High dose corticosteroid therapies for attacks and azathioprine, cyclophosphamide, interferon-α and anti-TNF agents for longterm preventive treatment are the general approach to NBD but trials are limited to build up a guideline [7]. All the accessible trials or manuscripts about biologic therapy in BD were included

Patient Characteristics in Our Study Population
Neuro-Behçet
Entero-Behçet
Major Vessel Disease
Joint Involvement
Findings
Conclusions
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