To explore the high risk factors for connective tissue diseases-associated pulmonary hypertension (CTD-PAH) by summarizing the clinical characteristics of CTD-PAH patients.

A total of 263 hospitalized patients with CTD whose pulmonary artery pressure was＞35 mmHg (1 mmHg=0.133 kPa), as measured by transthoracic echocardiography were enrolled at the Department of Rheumatology and Immunology, Nanjing Gulou Hospital from January 2003 to December 2017. Their clinical data including epidemiology, symptoms and signs, medications, and laboratory indexes were studied retrospectively.

Most CTD-PAH patients (77.19%, 203/263) were diagnosed at 19-65 years old, and most were female (83.7%, 220/263). Among all CTD-PAH cases, the proportion of systemic lupus erythematosus (SLE) was the highest (38%), followed by Sjogren's syndrome (29%) and systemic sclerosis (11%). The most common clinical manifestations related with PAH in CTD-PAH patients were chest tightness (77.57%), asthma (71.86%), and cough (61.98%). The most popular manifestations of primary CTD were Reynolds phenomenon (56.27%), dry mouth (47.53%), and rash (42.97%). The most common complications of CTD-PAH were interstitial lung disease (41.44%), cardiac insufficiency (32.32%), and proteinuria (25.48%). Hydroxychloroquine (62.36%) and cyclophosphamide (48.67%) were the most frequently used medications among immunosuppressants for CTD-PAH patients.

CTD-PAH predominantly occurs in middle-aged women, and SLE and Sjogren's syndrome are the most common. Reynolds phenomenon, dry mouth, rash, and interstitial lung disease are high risk factors for CTD-PAH.