title: Clinical and immunoserological characteristics of mixed connective tissue disease associated with pulmonary arterial hypertension.
abstract: We investigated the clinical characteristics and immunoserological alterations in patients with mixed connective tissue disease (MCTD) associated with pulmonary arterial hypertension (PAH). Anti-U1RNP autoantibodies, anti-endothelial cell antibodies (AECA) and serum thrombomodulin (TM) as well as von Willebrand factor antigen (vWFAg) concentrations were measured in 25 patients with MCTD associated with PAH and in 154 MCTD patients without PAH. The results showed that the probability of survival was lower in MCTD patients with PAH than in the 154 MCTD-non-PAH patients (5-year survival rate in MCTD with PAH: 73%, versus 96% in MCTD-non-PAH; P < 0.01). AECA were more frequently present in the sera of MCTD patients with PAH than in MCTD-non-PAH (P < 0.001). Serum TM and vWFAg levels were higher in MCTD-PAH patients than in MCTD-non-PAH patients (TM: P < 0.001; vWFAg: P < 0.001). Significant correlation was noticed between the quantity of AECA and TM level (r = 0.466) as well as the quantity of AECA and vWFAg level (r = 0.550). In conclusion, our results suggest that in MCTD the presence of AECA and endothelial cell activation may play a role in the development of PAH and in the maintenance of obliterative vascular processes.
authors.affiliation: 3rd Department of Internal Medicine, University of Debrecen, Medical and Health Science Centre, Debrecen, Hungary.
authors.forename: J
authors.initials: J
authors.lastname: Vegh
authors.forename: P
authors.initials: P
authors.lastname: Szodoray
authors.forename: J
authors.initials: J
authors.lastname: Kappelmayer
authors.forename: I
authors.initials: I
authors.lastname: Csipo
authors.forename: M
authors.initials: M
authors.lastname: Udvardy
authors.forename: G
authors.initials: G
authors.lastname: Lakos
authors.forename: M
authors.initials: M
authors.lastname: Aleksza
authors.forename: P
authors.initials: P
authors.lastname: Soltesz
authors.forename: A
authors.initials: A
authors.lastname: Szilágyi
authors.forename: M
authors.initials: M
authors.lastname: Zeher
authors.forename: G
authors.initials: G
authors.lastname: Szegedi
authors.forename: E
authors.initials: E
authors.lastname: Bodolay
mesh_terms: D000328:Adult; D017153:Antibodies, Anticardiolipin / Q000097:blood / Q000276:immunology; D000974:Antibodies, Antinuclear / Q000097:blood / Q000276:immunology; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000276:immunology / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D011651:Pulmonary Artery / Q000503:physiopathology*; D017412:Ribonucleoprotein, U1 Small Nuclear / Q000276:immunology; D016019:Survival Analysis
chemical_list: D017153:Antibodies, Anticardiolipin; D000974:Antibodies, Antinuclear; D001323:Autoantibodies; D017412:Ribonucleoprotein, U1 Small Nuclear; C087589:anti-endothelial cell antibody