Idiopathic and connective tissue disease-associated pulmonary arterial hypertension (PAH): Similarities, differences and the role of autoimmunity

IF 9.2 1区医学 Q1 IMMUNOLOGY Autoimmunity reviews Pub Date : 2024-01-03 DOI:10.1016/j.autrev.2024.103514

Elvira Favoino , Marcella Prete , Vasiliki Liakouli , Patrizia Leone , Adriana Sisto , Luca Navarini , Marta Vomero , Francesco Ciccia , Piero Ruscitti , Vito Racanelli , Roberto Giacomelli , Federico Perosa

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Abstract

Pre-capillary pulmonary arterial hypertension (PAH) is hemodynamically characterized by a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg, pulmonary capillary wedge pressure (PAWP) ≤15 mmHg and pulmonary vascular resistance (PVR) > 2. PAH is classified in six clinical subgroups, including idiopathic PAH (IPAH) and PAH associated to connective tissue diseases (CTD-PAH), that will be the main object of this review. The aim is to compare these two PAH subgroups in terms of epidemiology, histological and pathogenic findings in an attempt to define disease-specific features, including autoimmunity, that may explain the heterogeneity of response to therapy between IPAH and CTD-PAH.

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特发性和结缔组织病相关性肺动脉高压（PAH）：相似之处、不同之处以及自身免疫的作用

毛细血管前肺动脉高压（PAH）的血液动力学特征是平均肺动脉压（mPAP）≥20 mmHg，肺毛细血管楔压（PAWP）≤15 mmHg，肺血管阻力（PVR）≥2。PAH 可分为六个临床亚组，包括特发性 PAH（IPAH）和与结缔组织疾病相关的 PAH（CTD-PAH），这将是本综述的主要对象。本综述的主要目的是比较这两个 PAH 亚组在流行病学、组织学和病理学方面的发现，试图确定疾病的特异性特征（包括自身免疫），以解释 IPAH 和 CTD-PAH 对治疗反应的异质性。

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来源期刊

Autoimmunity reviews 医学-免疫学

CiteScore

24.70

自引率

4.40%

发文量

164

审稿时长

21 days

期刊介绍： Autoimmunity Reviews is a publication that features up-to-date, structured reviews on various topics in the field of autoimmunity. These reviews are written by renowned experts and include demonstrative illustrations and tables. Each article will have a clear "take-home" message for readers. The selection of articles is primarily done by the Editors-in-Chief, based on recommendations from the international Editorial Board. The topics covered in the articles span all areas of autoimmunology, aiming to bridge the gap between basic and clinical sciences. In terms of content, the contributions in basic sciences delve into the pathophysiology and mechanisms of autoimmune disorders, as well as genomics and proteomics. On the other hand, clinical contributions focus on diseases related to autoimmunity, novel therapies, and clinical associations. Autoimmunity Reviews is internationally recognized, and its articles are indexed and abstracted in prestigious databases such as PubMed/Medline, Science Citation Index Expanded, Biosciences Information Services, and Chemical Abstracts.