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The clinical diagnosis is nevertheless difficult because the disease manifestations vary widely, especially at the onset of disease. The aim of this study was to identify molecules specifically recognized by serum autoantibodies in patients with Beh\u00e7et's disease and to evaluate their diagnostic value. We screened a cDNA library from human microvascular endothelial cells with serum IgG from two patients with Beh\u00e7et's disease and isolated a reactive clone specific to the carboxy-terminal subunit of Sip1 (Sip1 C-ter). Using ELISA, we measured IgG, IgM and IgA specific to Sip1 C-ter in patients with various autoimmune diseases characterized by the presence of serum anti-endothelial cell antibodies, such as Beh\u00e7et's disease, systemic lupus erythematosus, systemic sclerosis and various forms of primary vasculitis, as well as in patients with diseases that share clinical features with Beh\u00e7et's disease, such as inflammatory bowel disease and uveitis. IgM immunoreactivity to Sip1 C-ter was significantly higher in patients with Beh\u00e7et's disease and in patients with primary vasculitis than in the other groups of patients and healthy subjects tested (<jats:italic>P<\/jats:italic> &lt; 10<jats:sup>-4<\/jats:sup> by Mann-Whitney test). ELISA detected IgG specific to Sip1 C-ter in sera from 11\/56 (20%) patients with Beh\u00e7et's disease, IgM in 23\/56 (41%) and IgA in 9\/54 (17%). No sera from patients with systemic lupus erythematosus, systemic sclerosis, inflammatory bowel disease, uveitis or healthy subjects but 45% of sera from patients with primary vasculitis contained IgM specific to Sip1 C-ter. Serum levels of soluble E-selectin, a marker of endothelial activation and inflammation, correlated with levels of serum IgM anti Sip-1 C-ter in patients with Beh\u00e7et's disease (r = 0.36, <jats:italic>P<\/jats:italic> = 0.023). In conclusion, Sip1 C-ter is a novel autoantigen in Beh\u00e7et's disease. IgM specific to Sip1 C-ter might be useful in clinical practice as an immunological marker of endothelial dysfunction in vasculitis.<\/jats:p>","DOI":"10.1186\/ar1940","type":"journal-article","created":{"date-parts":[[2006,4,20]],"date-time":"2006-04-20T14:30:09Z","timestamp":1145543409000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Identification and characterization of the carboxy-terminal region of Sip-1, a novel autoantigen in Beh\u00e7et's disease"],"prefix":"10.1186","volume":"8","author":[{"given":"Federica","family":"Delunardo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fabrizio","family":"Conti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paola","family":"Margutti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Cristiano","family":"Alessandri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Priori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandra","family":"Siracusano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachele","family":"Rigan\u00f2","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisabetta","family":"Profumo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guido","family":"Valesini","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maurizio","family":"Sorice","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elena","family":"Ortona","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2006,4,12]]},"reference":[{"key":"1818_CR1","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1016\/j.berh.2004.02.008","volume":"18","author":"SE Marshall","year":"2004","unstructured":"Marshall SE: Beh\u00e7et's disease. 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