One year in review 2018: Behçet's syndrome

New epidemiologic studies from Poland, Jordan, Algeria, Taiwan and Korea highlight the geographic differences in incidence, prevalence and clinical features of Behçet's syndrome (BS). A study from Austria comparing clinical manifestations of their BS patients with different countries of origin suggest that environmental factors may be important in the disease phenotype of BS. New genetic association studies dealing with the innate and acquired aspects of BS prevailed during 2017 and novel susceptibility and regulatory factors were described. Common denominators among various disease processes were again highlighted and epigenetic factors were emphasised. "Bagel sign" pattern, a central lesion with hypo-intense core and hyper-intense rim was defined in the spinal MRIs of the patients with neuro-BS especially during the acute attacks of myelopathy. This distinctive pattern suggests venous thrombosis and surrounding oedema in the spinal cord. Pseudotumour cerebri may present with similar clinical presentation to that observed in cerebral venous sinus thrombosis, responds well to immunosuppressive treatment, and could be associated with venous thrombotic relapses. Menstruation and certain food appear to exacerbate skin and mucosa lesions in BS. The EULAR recommendations for the treatment of BS have been updated with 5 new overarching principles and one additional recommendation for surgical management of vascular complications. Infliximab initiated earlier in the course of uveitis yields a better visual outcome. Tapering or stopping of anti-TNF agents seem to be possible when remission has been achieved. Adalimumab appears to be more effective for venous thrombosis than classical immunosuppressives. Oral anticoagulants might not be crucial for cerebral or peripheral venous thrombosis. Transcatheter embolisation of pulmonary aneurysms may be life-saving by providing immediate control of haemoptysis. The results of surgery for pulmonary artery involvement appear to be satisfactory.