With an unknown etiology, pathogenesis and protean manifestations, Behçet's Syndrome (BS) remains a true construct – disease. As such, formulating disease criteria for BS is no easy task. This difficulty is compounded by the unfortunately much in vogue concept of formulating separate disease critera for classification and diagnosis (1). There are, however, specific and not so-specific elements of BS as a construct – disease and eye involvement is a specific element, as a well designed recent study underlined (2).
Perhaps the more important contributions related to the understanding the pathogenesis within the past year were the papers related to the activations of the JAK-STAT and the NOTCH pathways in BS (3,4) with the upregulation SOCS proteins which negatively regulate the JAK-STAT pathway (5). HLA B51 remains the most prominent genetic association of BS and a recent work shed more light on its role in antigen recognition (6). Also in an important epigenetic study there were aberrations in the DNA methylation of genes related to cycoskletal function in BS and these tended to reverse with therapy (7).
Some features of BS tend to cluster and vascular disease, a major cause of morbidity and mortality, is one such cluster. In a recent analysis we attempted to better define this cluster which involves dural sinus thrombi-pulmonary vascular disease-vena caval disease- peripheral deep vein thrombosis and finally superficial vein thrombosis (8). It is interesting to note that arterial disease, mainly on the form of anuerysms (excluding those in the pulmonary tree) is outside this cluster. We have also recently learned that the Budd-Chiari Syndrome, a relatively rare but a very serious complication of BS, can also present in milder forms, if specifically searched for (9).
Our management know-how and capabilities are also improving. The precise role of colchicine in treatment and the use of anticoagulants in thrombotic disease in BS have, for years, been two important issues of debate. Within the past year two new observational studies provided additional useful data. Long term, 17 years, follow up of a group of patients from a double blind study of colchicine against placebo showed that the earlier use of colchicine did not have any affect on long term emergence of major morbidities as assessed by subsequent immunosuppresive use (10). For the role of anticoagulation, a retrospective reevaluation of management of vascular disease among 935 patients from 15 centers in Turkey revealed that the addition of anticoagulation to immunosuppresives was not effective in preventing recurrences (11). It is, however, to be underlined that a properly controlled randomized withdrawal study is still needed in the case of colchicine and a traditional randomized controlled trial in the case of the potential role of anticoagulants. Within the past year there were also a number of observational studies related to the successful use of biologics (12,13,14). Finally, a double blind, placebo controlled study with apremilast, an oral phosphodiesterase inhibitor, among patients with mainly skin-mucosa disease showed good effect of this agent in oral and, among the relatively small number of patients, in genital ulceration (15). Possible beneficial effects of apremilast on eye, vascular and central nervous disease remain to be seen.
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Disclosure of Interest None declared