ABSTRACT

Introduction

Pulmonary arterial hypertension (PAH), also referred to as group 1 pulmonary hypertension, occurs either primarily or in association with other diseases such as connective tissue diseases (CTD). Of CTD, systemic sclerosis (SSc), systemic lupus erythematosus and mixed connective tissue disease are commonly accompanied with PAH. It is of note that SSc-PAH is associated with distinctive histopathology, an unfavorable outcome, and a blunted responsiveness to modern PAH therapies.

Areas covered

The data in articles published until May 2020 in peer-reviewed journals, covered by PubMed databank, are discussed. The current review introduces recent advances over the past years which have moved our understanding of CTD-PAH forward and discusses what we are currently able to do and what will be necessary in the future to overcome the yet unsatisfactory situation in the management of CTD-PAH, particularly in that of SSc-PAH.

Expert opinion

A multifaceted and integrated approach would be crucial to improve the outcome of patients with SSc-PAH. The authors also propose a possible algorithm to classify and treat SSc patients with suspicion of pulmonary vascular disease.

中文翻译：

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结缔组织病相关肺动脉高压的诊断和预后标志物及治疗：目前的建议和最新进展

摘要

介绍

肺动脉高压 (PAH)，也称为第 1 组肺动脉高压，主要发生或与其他疾病如结缔组织病 (CTD) 相关。在CTD中，系统性硬化症（SSc）、系统性红斑狼疮和混合性结缔组织病常伴有PAH。值得注意的是，SSc-PAH 与独特的组织病理学、不利的结果以及对现代 PAH 疗法的反应迟钝有关。

覆盖区域

讨论了 2020 年 5 月之前在 PubMed 数据库涵盖的同行评审期刊上发表的文章中的数据。本综述介绍了过去几年的最新进展，这些进展推动了我们对 CTD-PAH 的理解，并讨论了我们目前能够做什么以及未来需要做什么来克服 CTD-PAH 管理中尚不令人满意的情况，特别是在 SSc-PAH 中。

专家意见

多方面的综合方法对于改善 SSc-PAH 患者的预后至关重要。作者还提出了一种可能的算法来对疑似肺血管疾病的 SSc 患者进行分类和治疗。