Université Paris-Saclay, School of Medicine, Le Kremlin-Bicêtre, France; INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis-Robinson, France; AP-HP, Department of Respiratory and Intensive CareMedicine, Hôpital Bicêtre, Le Kremlin-Bicêtre, France.
Pathology Department, University Hospital of Giessen and Marburg, Rudolf-Buchheim-Straße 8, Gießen 35392, Deutschland; German Center for Lung Research (DZL), Giessen, Germany.
Clin Chest Med. 2024 Mar;45(1):185-197. doi: 10.1016/j.ccm.2023.08.007. Epub 2023 Sep 1.
Pulmonary hypertension is a life-threatening complication of advanced sarcoidosis. Many mechanisms can cause an elevation of pulmonary pressure in sarcoidosis, leading to precapillary or postcapillary pulmonary hypertension. Sarcoidosis-associated pulmonary hypertension contributes to severe exertional dyspnea, reduced exercise capacity, and notably compromised the survival. Despite the critical functional and prognostic implications of pulmonary hypertension in sarcoidosis, there is a scarcity of specific guidelines on the management of these patients due to a lack of evidence. Hence, further research is required to identify subgroups of patients who may benefit from pulmonary arterial hypertension-targeted therapies and/or immunosuppressive therapies.
肺动脉高压是晚期肉样瘤病的一种危及生命的并发症。许多机制可导致肉样瘤病中的肺压升高,导致毛细血管前或毛细血管后肺动脉高压。肉样瘤病相关性肺动脉高压导致严重的运动性呼吸困难、运动能力下降,显著降低生存率。尽管肺动脉高压对肉样瘤病的功能和预后有重要影响,但由于缺乏证据,这些患者的管理缺乏具体的指南。因此,需要进一步的研究来确定可能受益于肺动脉高压靶向治疗和/或免疫抑制治疗的患者亚组。