Pulmonary hypertension in systemic lupus erythematosus: a systematic review and analysis of 642 cases in Chinese population

Pulmonary hypertension (PH) is an increasingly recognized complication of systemic lupus erythematosus (SLE). To develop a more comprehensive understanding of the clinical and pathological characteristics of pulmonary hypertension associated with systemic lupus erythematosus (PH/SLE) in the Chinese population, a systematic review of the literature up to 2012 was conducted. Six hundred and forty-two Chinese PH/SLE cases from 22 studies were identified as well documented and further analyzed. Transthoracic echocardiography (TTE), X-ray, electrocardiogram and right heart catheterization (RHC) were performed to diagnose PH in SLE patients. The mean age of subjects was 35.5 years, the male to female ratio was 1:14, and the mean duration of SLE when PH was diagnosed was 10.7 years. The prevalence of PH in SLE was 2.8–23.3 %. Symptoms were usually nonspecific, and the observed clinical characteristics include Raynaud’s phenomenon (41.4 %), serous effusion (27.7 %), positive RNP (51.5 %) and positive ACL (46.6 %). Gold standard RHC is strongly recommended, especially for those who had resting pulmonary arterial systolic pressure >30 mmHg on TTE with the aforementioned clinical characteristics. Corticosteroids, immunosuppressants and vasodilators were the most common medications employed in treatment. Early identification and standard PH treatment with intensive SLE treatment can improve the prognosis.