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The purpose of this study was to determine whether patients with primary pulmonary hypertension (PPH) and appetite\u2010suppressant\u2010associated PPH have anti\u2010Fbn\u20101 autoantibodies. In addition we assessed the human leucocyte antigen (HLA) class II alleles (DRB1, 3, 4, 5 and DQB1) in these patients in order to determine whether the response is genetically restricted. The frequency of anti\u2010Fbn\u20101 autoantibodies in patient groups was compared with that of a control group of 88 healthy patients, and HLA was correlated similarly with a group of 51 healthy subjects. Anti\u2010Fbn\u20101 autoantibodies were found at high frequency in PPH: in 70 of 75 adults with PPH (93%), in 28 of 33 children with PPH (84.8) and in 12 of 18 (67%) patients with appetite\u2010suppressant\u2010associated PPH. Utilization of two Fbn\u20101 fusion proteins allowed us to determine the dominant determinant region, recognized by anti\u2010Fbn\u20101 autoantibodies, which may be located on the N\u2010terminal fragment of the Fbn\u20101 protein. No significant immunogenetic correlations were found when the PPH patient groups were compared with normal controls. This novel category of autoantibodies is found in diseases characterized by endothelial and extracellular matrix protein alterations and fibrosis.<\/jats:p>","DOI":"10.1046\/j.1365-3083.2000.00733.x","type":"journal-article","created":{"date-parts":[[2003,3,12]],"date-time":"2003-03-12T20:14:23Z","timestamp":1047500063000},"page":"607-611","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":28,"title":["Fine Specificity of Anti\u2010Fibrillin\u20101 Autoantibodies in Primary Pulmonary Hypertension Syndrome"],"prefix":"10.1111","volume":"51","author":[{"family":"Morse","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Antohi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Kasturi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Saito","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Fotino","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Humbert","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Simonneau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Basst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"family":"Bona","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2001,12,25]]},"reference":[{"key":"e_1_2_5_2_2","doi-asserted-by":"publisher","DOI":"10.1378\/chest.104.1.236"},{"key":"e_1_2_5_3_2","doi-asserted-by":"publisher","DOI":"10.1161\/01.CIR.95.12.2603"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1038\/ng0397-277"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0735-1097(86)80301-1"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0065-2776(08)60836-6"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(92)90238-7"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2249.1996.d01-848.x"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1164\/ajrccm.155.1.9001324"},{"key":"e_1_2_5_10_2","first-page":"1688","article-title":"Antibodies of fibrin bind tissue type plasminogen activator in systemic sclerosis","volume":"22","author":"Fritzler MJ","year":"1995","journal-title":"J Rheumatol"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12339373"},{"key":"e_1_2_5_12_2","doi-asserted-by":"publisher","DOI":"10.1164\/arrd.1984.129.5.850"},{"key":"e_1_2_5_13_2","doi-asserted-by":"publisher","DOI":"10.1016\/0008-8749(83)90223-X"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.3109\/08916939808996283"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-115-5-343"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780251101"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780230510"},{"key":"e_1_2_5_18_2","unstructured":"RosnerBA. 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