New Evidence-Based Treatment Approach in Behçet's Disease

Abstract

Behçet's disease (BD) is a chronic, relapsing, and debilitating systemic vasculitis of unknown aetiology with the clinical features of mucocutaneous lesions, ocular, vascular, articular, neurologic, gastrointestinal, urogenital, and pulmonary involvement. The disease is much more frequent along the ancient “Silk Route” extending from Eastern Asia to the Mediterranean basin, compared with Western countries. The disease usually starts around the third or fourth decade of life. Male sex and a younger age of onset are associated with more severe disease. Although the treatment has become much more effective in recent years, BD is still associated with severe morbidity and considerable mortality. The main aim of the treatment should be the prevention of irreversible organ damage. Therefore, close monitoring, early, and appropriate treatment is mandatory to reduce morbidity and mortality. The treatment is mainly based on the suppression of inflammatory attacks of the disease using immunomodulatory and immunosuppressive agents. In this paper, current state of knowledge regarding the therapeutic approaches is outlined. To provide a rational framework for selecting the appropriate therapy along the various treatment choices, a stepwise, symptom-based, evidence-based algorithmic approach was developed.

HLA-B51 subtypes in Turkish patients with Behçet's disease and their correlation with clinical manifestations

“…Considering the clinical findings in the BD patients, 100% had OUs, 82.4% had GUs, 47.1% had joint involvement, and 35.3% had ocular involvement and EN, consistent with previous studies (Tursen et al, 2003;Alpsoy et al, 2007Alpsoy et al, , 2012. The GUs were more frequent in the female patients, which is consistent with an earlier study, in which the clinical findings of 2313 patients were analyzed (Tursen et al, 2003).…”

Anticoagulation of Behcet’s Disease with Thrombosis: A Series of 28 Cases in a Tertiary Care Centre in Saudi Arabia

“…Vascular thrombosis occurred more in male patients. Male sex and a younger age of onset usually associated with more severe diseases [9].…”

Behçet's disease: A comprehensive review with a focus on epidemiology, etiology and clinical features, and management of mucocutaneous lesions

“…All therapy should be combined with bedrest. 26,66 However, it is very important for clinicians to be aware that topical treatment has a great possibility of having only local effects and should almost always be associated with systemic therapy.…”

“…The treatment algorithm for mucocutaneous BD summarized below are mainly based on controlled studies. 66,67 In Figure 3, I have tried to recommend the most appropriate treatment we have already used in our clinical practice. In the treatment of OU, GU, EN-like lesions and PPL, colchicine should be the first choice.…”

“…Almost all trials with anti-TNF-a agents, infliximab, adalimumab and etanercept reported encouraging results for recalcitrant mucocutaneous lesions besides ocular and gastrointestinal symptoms, arthritis and cerebral vasculitis. 66,115 Other biologics such as IL-1, -2, -6, -12, -17 and -23-blocking agents and monoclonal antibody against CD20 have been increasingly used in recent years in patients with BD refractory to other treatments. Most of the studies related to the respected biologics concentrated on the eye disease or other major organ involvements.…”