Clinical and Immunoserological Characteristics of Mixed Connective Tissue Disease Associated with Pulmonary Arterial Hypertension

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 vo...

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Bibliographic Details
Published in:Scandinavian journal of immunology 2006-07, Vol.64 (1), p.69-76
Main Authors: Vegh, J., Szodoray, P., Kappelmayer, J., Csipo, I., Udvardy, M., Lakos, G., Aleksza, M., Soltesz, P., Szilágyi, A., Zeher, M., Szegedi, G., Bodolay, E.
Format: Article
Language:English
Subjects:
Adult
Antibodies, Anticardiolipin - blood
Antibodies, Anticardiolipin - immunology
Antibodies, Antinuclear - blood
Antibodies, Antinuclear - immunology
Autoantibodies - blood
Autoantibodies - immunology
Echocardiography, Doppler
Female
Humans
Hypertension, Pulmonary - etiology
Hypertension, Pulmonary - immunology
Hypertension, Pulmonary - physiopathology
Male
Middle Aged
Mixed Connective Tissue Disease - complications
Mixed Connective Tissue Disease - immunology
Mixed Connective Tissue Disease - physiopathology
Pulmonary Artery - physiopathology
Ribonucleoprotein, U1 Small Nuclear - immunology
Survival Analysis
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Summary: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 
ISSN:0300-9475
1365-3083
DOI:10.1111/j.1365-3083.2006.01770.x