CAJ | 학술논문

随着对结缔组织病相关性间质性肺疾病(CTD-ILD)研究的不断深入,CTD-ILD继发肺动脉高压(pulmonary hypertension,PH)较无伴发肺间质病变的风湿结缔组织病、特发性肺间质纤维化发病率高,而PH作为间质性肺疾病及结缔组织病的并发症,往往提示预后不良.CTD-ILD继发PH较为常见,且预后差,已经引起学术界的广泛关注,并对其进行了深入的研究,但目前尚无突破性进展.目前存在的主要问题包括发病机制复杂、病情多呈进行性进展、临床缺乏早期诊断手段、治疗措施有限等.而早期诊断、早期干预可望改善患者的生活质量、延长生存期.本文阐述了CTD-ILD的发病机制、诊断、治疗方面的研究现状,以深化对CTD-ILD继发PH的认识,为临床提供诊断、降低肺动脉压、抗纤维化的治疗思路.
수착대결체조직병상관성간질성폐질병(CTD-ILD)연구적불단심입,CTD-ILD계발폐동맥고압(pulmonary hypertension,PH)교무반발폐간질병변적풍습결체조직병、특발성폐간질섬유화발병솔고,이PH작위간질성폐질병급결체조직병적병발증,왕왕제시예후불량.CTD-ILD계발PH교위상견,차예후차,이경인기학술계적엄범관주,병대기진행료심입적연구,단목전상무돌파성진전.목전존재적주요문제포괄발병궤제복잡、병정다정진행성진전、림상결핍조기진단수단、치료조시유한등.이조기진단、조기간예가망개선환자적생활질량、연장생존기.본문천술료CTD-ILD적발병궤제、진단、치료방면적연구현상,이심화대CTD-ILD계발PH적인식,위림상제공진단、강저폐동맥압、항섬유화적치료사로.
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.