期刊
EUROPEAN JOURNAL OF CLINICAL INVESTIGATION
卷 48, 期 2, 页码 -出版社
WILEY
DOI: 10.1111/eci.12876
关键词
connective tissue diseases; pulmonary arterial hypertension; pulmonary hypertension; systemic lupus erythematosus; systemic sclerosis
Recent studies have clarified that pulmonary arterial hypertension associated with connective tissue diseases (CTD-PAH) has some distinctive clinical aspects from other PAH, such as high prevalence, venous and cardiac involvement, less favourable outcome, helpfulness of detection algorithm, response to immunosuppression, pre-PAH conditions in borderline pulmonary arterial pressure and coexistence of interstitial lung disease. In this review, by focusing on these distinctive aspects, we discuss how to provide an efficacious and safe management of CTD-PAH and garner attention to areas where further evidence is desired.
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