Fine Specificity of Anti-Fibrillin-1 Autoantibodies in Primary Pulmonary Hypertension Syndrome
Autor: | Jane H. Morse, Shinichiro Saito, Constantin A. Bona, K Kasturi, S Antohi, Marc Humbert, R J Basst, G. Simonneau, M Fotino |
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Rok vydání: | 2000 |
Předmět: |
Adult
Male Fibrillin-1 Hypertension Pulmonary Immunology Human leukocyte antigen Fibrillins Mice Mixed connective tissue disease Antibody Specificity Calcinosis Fibrosis medicine Animals Humans Allele Child Alleles Autoantibodies Mice Knockout business.industry Histocompatibility Testing Microfilament Proteins Histocompatibility Antigens Class II Autoantibody Syndrome General Medicine medicine.disease Pulmonary hypertension Mice Inbred C57BL Female business Fibrillin |
Zdroj: | Scandinavian Journal of Immunology. 51:607-611 |
ISSN: | 1365-3083 0300-9475 |
Popis: | Autoantibodies to fibrillin-1 (Fbn-1) have been found in systemic sclerosis (SSc), calcinosis, Raynaud's esophagael dysmotility, sclerodectyly, and telaengectasia (CREST) and mixed connective tissue disease (MCTD) diseases. The purpose of this study was to determine whether patients with primary pulmonary hypertension (PPH) and appetite-suppressant-associated PPH have anti-Fbn-1 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-Fbn-1 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-Fbn-1 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-suppressant-associated PPH. Utilization of two Fbn-1 fusion proteins allowed us to determine the dominant determinant region, recognized by anti-Fbn-1 autoantibodies, which may be located on the N-terminal fragment of the Fbn-1 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. |
Databáze: | OpenAIRE |
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