Clinical analysis of 79 pulmonary arterial hypertension cases from 1892 connective tissue disease patients

Abstract

To understand the prevalence, investigate the correlation of clinical features, explore the early-stage diagnosis and treatment of pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD). All cases with pulmonary arterial hypertension in 1892 CTD patients were analyzed retrospectively. The risk factor of PAH was evaluated and the prognostic influence of different treatments and primary diseases analyzed. The prevalence of PAH in patients with connective tissue disease was about 4.2%(79/1892). In these patients, systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) had the highest incidence of PAH (18.18% and 12.00%) (P < 0.01). It was obviously higher than polymyositis/dermatomyositis (6.2%), systemic lupus erythematosus (4.4%), Sjogren syndrome (3.8%), rheumatoid arthritis (0.8%) and anti-phospholipid syndrome (0.5%), etc. (P < 0.01). Raynaud's phenomenon was related to a higher pulmonary arterial pressure (P < 0.01). There was a positive correlation (P < 0.01) between the presence of Raynaud's phenomenon and pulmonary arterial pressure. Abnormal lung function was a common finding. There were associations (P < 0.05) between the degree of pulmonary hypertension and IgG, anti-U1RNP antibody positive, antiphospholipid antibody positive, pericardial effusion and interstitial pneumonia. PAH is common in connective tissue disease. SSc and MCTD have the highest prevalence of PAH. The presence of Raynaud's phenomenon anti-U1RNP antibody is positively correlated with pulmonary arterial pressure. It can predict the development of PAH. It is useful to perform ultraechocardiogrphy for an early-stage diagnosis and prognostic analysis.