Connective tissue disease related interstitial lung disease complicating pulmonary hypertension: a respective review

Abstract

With the deepening of the study on connective tissue disease related interstitial lung disease (CTD-ILD),the prevalence rate of CTD-ILD complicating pulmonary hypertension (PH) is higher than that of the simple connective tissue disease or idiopathic pulmonary fibrosis.CTD-ILD complicating PH is common,and the prognosis is poor,which has caused widespread concern in academic circles,and has carried on the thorough research,but there has no breakthrough.At present the main problems include complex pathogenesis,progressive progression,lack of early diagnosis means,and limited therapeutic measures.The early diagnosis and early intervention are expected to improve the patients' quality of life and prolong survival.This paper expounds the research status of pathogenesis,diagnosis,and treatment of CTD-ILD,in order to deepen the understanding of CTD-ILD complicating PH. Key words: Interstitial lung disease; Connective tissue disease; Pulmonary hypertension