Successful Treatment of Refractory Behçet's Disease with the TNF-α Blocker Infliximab

Behçet's disease (BD) is an autoimmune, chronically relapsing, multisystemic, inflammatory disorder, classified among the vasculitides (1). So far, the pathogenesis of BD is not completely understood but most of the symptoms of the disease are attributed to small-vessel vasculitis. BD is prevalent in the Middle East, Asia, Japan and is rare among Caucasians. The disease affects more men than women and tends to develop mainly in the third to fourth decade of life (2). As there are no pathognomonic laboratory parameters, the diagnosis is suspected by the simultaneous or sequenced appearance of certain symptoms. According to the International Study Group criteria for BD, these include the presence of oral aphthae (at least three times in one year) plus any of two of the following symptoms: recurrent genital aphthae, ocular lesions (uveitis), skin lesions (erythema nodosum, pseudofolliculitis), positive pathergy test and/or organ involvement (lung, joints, brain and/or gastrointestinal tract). The frequencies of these symptoms were published by the “Behçet’s syndrome research committee of Japan” and are summarized in Table 1. A number of heritable risk factors for BD have been reported (1). These include among others HLA-haplotypes B51 (positive in more than 60% of patients), 12 and 27; polymorphisms in gene loci encoding certain complement proteins, tumor necrosis factor- (TNF-), members of the heat shock protein family (HSP) as well as the MHC class I chain-related proteins (MIC); factor V Leiden mutation resulting in venous occlusion; intercellular adhesion molecule (ICAM) polymorphisms and, abnormalities in killing inhibitory receptors (KIR) (3).