Morimatsu Yoshitaka, Tahara Nobuhiro, Okamoto Masaki, Bekki Munehisa, Tahara Atsuko, Eto Yoshiko, Kugai Tadahiro, Koga Yuki, Maeda-Ogata Shoko, Honda Akihiro, Igata Sachiyo, Zaizen Yoshiaki, Tanoue Shuichi, Hoshino Tomoaki, Ishitake Tatsuya, Fukumoto Yoshihiro
Department of Environmental Medicine, Kurume University School of Medicine, Kurume 830-0011, Japan.
Division of Cardiovascular Medicine, Department of Medicine, Kurume University School of Medicine, Kurume 830-0011, Japan.
Medicina (Kaunas). 2025 Feb 14;61(2):342. doi: 10.3390/medicina61020342.
Sarcoidosis is a granulomatous disorder of unknown etiology characterized by multisystem non-caseating granulomas. Pulmonary hypertension (PH) is a well-known complication of sarcoidosis and is associated with increased morbidity and mortality. The actual epidemiology of sarcoidosis-associated PH (SAPH) remains unknown, and its pathogenesis has not been fully elucidated. SAPH is classified under the miscellaneous category (group 5 of the PH classification). The clinical presentation of SAPH is variable and not always proportional to the severity of sarcoidosis. Appropriate management for SAPH by an experienced physician is important; however, no treatment algorithm for SAPH has been established. Lung transplantation should be considered in refractory cases. Pulmonary arterial hypertension-specific vasodilators targeting the endothelin pathway, nitric oxide pathway, and prostacyclin pathway have improved the clinical functions and hemodynamics in some patients with SAPH.
结节病是一种病因不明的肉芽肿性疾病,其特征为多系统非干酪样肉芽肿。肺动脉高压(PH)是结节病的一种众所周知的并发症,与发病率和死亡率增加相关。结节病相关肺动脉高压(SAPH)的实际流行病学情况仍不清楚,其发病机制也尚未完全阐明。SAPH被归类于混合性类别(PH分类中的第5组)。SAPH的临床表现各异,且并不总是与结节病的严重程度成正比。由经验丰富的医生对SAPH进行恰当管理很重要;然而,尚未建立针对SAPH的治疗方案。难治性病例应考虑肺移植。针对内皮素途径、一氧化氮途径和前列环素途径的肺动脉高压特异性血管扩张剂已改善了一些SAPH患者的临床功能和血流动力学。