Department of Rheumatology, Hacettepe University, Faculty of Medicine, Ankara, Türkiye.
Department of Rheumatology, Etlik City Hospital, Ankara, Türkiye.
Anatol J Cardiol. 2023 Dec 1;27(12):677-687. doi: 10.14744/AnatolJCardiol.2023.3650. Epub 2023 Nov 21.
The primary systemic vasculitides are rare diseases characterized by vessel wall inflammation. Isolated pulmonary vasculitis, large-vessel vasculitis, and Behçet's disease are mimickers of chronic thromboembolic pulmonary hypertension (CTEPH); group IV pulmonary hypertension (PH) can occur as a devastating complication in the course of these diseases. Pulmonary endarterectomy, balloon angioplasty, anticoagulation and pulmonary vasodilator agents are the main treatment options for CTEPH. There is no specific recommendation for the treatment of patients having group IV PH due to primary systemic vasculitides. We reviewed herein data about group IV PH due to primary systemic vasculitides.
原发性系统性血管炎是一种以血管壁炎症为特征的罕见疾病。孤立性肺血管炎、大血管血管炎和贝赫切特病是慢性血栓栓塞性肺动脉高压（CTEPH）的模拟疾病；这些疾病的病程中可能会发生 IV 组肺动脉高压（PH）这一灾难性并发症。肺动脉血栓内膜切除术、球囊血管成形术、抗凝和肺血管扩张剂是 CTEPH 的主要治疗选择。对于由原发性系统性血管炎引起的 IV 组 PH 患者，目前尚无特定的治疗建议。我们在此回顾了与原发性系统性血管炎相关的 IV 组 PH 的相关数据。
