Demerouti Eftychia, Tsangaris Iraklis, Dimitroulas Theodoros, Giannakoulas George, Katsimpri Pelagia, Mitrouska Ioanna, Orfanos Stylianos, Skoularigkis Ioannis, Voulgari Paraskevi, Sfikakis Petros
Onassis Cardiac Surgery Center, Pulmonary Hypertension Unit, Athens, Greece.
Attikon University Hospital, Pulmonary Hypertension Clinic, Athens, Greece.
Mediterr J Rheumatol. 2019 Jun 29;30(2):90-93. doi: 10.31138/mjr.30.2.90. eCollection 2019 Jun.
Pulmonary Arterial Hypertension is regarded as a devastating disease, complicating Connective Tissue Diseases. Although much progress has been achieved in the last 20 years, several unfulfilled needs in diagnosis and management of PAH in these patients may still be identified. After a systematic review of the literature and integrating results from the latest research articles, key clinical issues for the screening and diagnosis of Pulmonary Arterial Hypertension in Connective Tissue Disorder Patients and specifically Scleroderma patients are described in this article, allowing physicians to contribute to early diagnosis of patients with Scleroderma-associated Pulmonary Arterial Hypertension.
肺动脉高压被视为一种毁灭性疾病,是结缔组织病的并发症。尽管在过去20年里已经取得了很大进展,但在这些患者的肺动脉高压诊断和管理方面仍有一些未满足的需求。在对文献进行系统综述并整合最新研究文章的结果后,本文描述了结缔组织病患者尤其是硬皮病患者肺动脉高压筛查和诊断的关键临床问题,以便医生能够促进硬皮病相关肺动脉高压患者的早期诊断。