Kato Masaru, Sugimoto Ayako, Atsumi Tatsuya
Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo, Japan.
First Department of Medicine, Hokkaido University Hospital , Sapporo, Japan.
Expert Rev Clin Immunol. 2020 Oct;16(10):993-1004. doi: 10.1080/1744666X.2021.1825940. Epub 2020 Oct 5.
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.
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.
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.
肺动脉高压（PAH），也称为1型肺动脉高压，可原发性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相关的PAH具有独特的组织病理学特征、不良预后，并且对现代PAH治疗的反应不敏感。
讨论了截至2020年5月在同行评审期刊上发表的、被PubMed数据库收录的文章中的数据。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相关PAH的认识，并讨论了我们目前能够做什么以及未来需要做什么，以克服CTD相关PAH管理中仍不尽人意的状况，特别是SSc相关PAH的管理。
多方面综合方法对于改善SSc相关PAH患者的预后至关重要。作者还提出了一种可能的算法，用于对怀疑患有肺血管疾病的SSc患者进行分类和治疗。
