PMID- 27886800 OWN - NLM STAT- MEDLINE DCOM- 20170816 LR - 20181202 IS - 1532-1770 (Electronic) IS - 1521-6942 (Linking) VI - 30 IP - 2 DP - 2016 Apr TI - Behcet's disease: How to diagnose and treat vascular involvement. PG - 279-295 LID - S1521-6942(16)30042-0 [pii] LID - 10.1016/j.berh.2016.08.002 [doi] AB - Behcet's disease is a multisystem disorder with unknown etiology and a unique geographic distribution. The disease is characterized by recurrent skin-mucosa lesions and sight-threatening panuveitis. Vascular involvement, which is more common and more severe among males, has also equally characteristic features such as affecting mostly veins, having a significant tendency for thrombosis, and running a relapsing course. Lower extremity vein thrombosis (LEVT) is the most frequent manifestation, followed by vena cava inferior thrombosis. Pulmonary artery involvement (PAI), the most common form of arterial involvement, manifests as aneurysms and "in situ" thrombosis. PAI and Budd-Chiari syndrome are the leading causes of increased mortality. In vascular cluster, typically, several types of venous or arterial vascular involvement may accumulate in the same individual. LEVT or cerebral venous sinus thrombosis is often present in these subgroups as the first event. Immunosuppressive treatment is essential in preventing the attacks and increasing survival. CI - Copyright A(c) 2016 Elsevier Ltd. All rights reserved. FAU - Seyahi, Emire AU - Seyahi E AD - University of Istanbul, Cerrahpasa Medical Faculty, Department of Medicine, Division of Rheumatology, Istanbul 81310, Turkey. Electronic address: eseyahi@yahoo.com. LA - eng PT - Journal Article PT - Review DEP - 20160916 PL - Netherlands TA - Best Pract Res Clin Rheumatol JT - Best practice & research. Clinical rheumatology JID - 101121149 RN - 0 (Immunosuppressive Agents) SB - IM MH - Adult MH - Behcet Syndrome/complications/*diagnosis/drug therapy MH - Humans MH - Immunosuppressive Agents/therapeutic use MH - Male MH - Thrombosis/etiology OTO - NOTNLM OT - Anticoagulation OT - Azathioprine OT - Behcet's disease OT - Lower extremity vein thrombosis OT - Pulmonary artery aneurysms OT - Thrombosis OT - Vasculitis OT - Vena cava thrombosis EDAT- 2016/11/26 06:00 MHDA- 2017/08/17 06:00 CRDT- 2016/11/26 06:00 PHST- 2016/06/07 00:00 [received] PHST- 2016/07/27 00:00 [revised] PHST- 2016/07/27 00:00 [accepted] PHST- 2016/11/26 06:00 [entrez] PHST- 2016/11/26 06:00 [pubmed] PHST- 2017/08/17 06:00 [medline] AID - S1521-6942(16)30042-0 [pii] AID - 10.1016/j.berh.2016.08.002 [doi] PST - ppublish SO - Best Pract Res Clin Rheumatol. 2016 Apr;30(2):279-295. doi: 10.1016/j.berh.2016.08.002. Epub 2016 Sep 16.