结缔组织病相关肺动脉高压(CTD-PAH)是继发肺动脉高压(PAH)中最常见的临床类型,较原发PAH更为多见。K urasaw a认为CTD-PAH并非内科常见病,但是结缔组织病(CTD)的严重并发症;未经恰当处置的CTD-PAH患者2 a病死率可达45%~60%。死亡原因主要为心功能衰竭、呼吸功能衰竭和严重感染 Connective tissue disease-associated pulmonary hypertension (CTD-PAH) is the most common clinical type of secondary pulmonary hypertension (PAH), more common than primary PAH. Kurasawa argues that CTD-PAH is not a common medical condition but a serious complication of connective tissue disease (CTD); 2-year mortality rates of up to 45% to 60% in untreated CTD-PAH patients. The main causes of death were heart failure, respiratory failure and severe infection