Université Paris-Saclay; INSERM UMR_S 999; Assistance Publique Hôpitaux de Paris, Service de Pneumologie et Soins Intensifs Respiratoires, Hôpital Bicêtre, Le Kremlin Bicêtre, France.
Dept of Cardiology, St. Antonius Hospital, Nieuwegein and University Medical Center Utrecht, Utrecht, The Netherlands.
Eur Respir Rev. 2022 Feb 9;31(163). doi: 10.1183/16000617.0165-2021. Print 2022 Mar 31.
Sarcoidosis-associated pulmonary hypertension (SAPH) is an important complication of advanced sarcoidosis. Over the past few years, there have been several studies dealing with screening, diagnosis and treatment of SAPH. This includes the results of two large SAPH-specific registries. A task force was established by the World Association of Sarcoidosis and Other Granulomatous disease (WASOG) to summarise the current level of knowledge in the area and provide guidance for the management of patients. A group of sarcoidosis and pulmonary hypertension experts participated in this task force. The committee developed a consensus regarding initial screening including who should undergo more specific testing with echocardiogram. Based on the results, the committee agreed upon who should undergo right-heart catheterisation and how to interpret the results. The committee felt there was no specific phenotype of a SAPH patient in whom pulmonary hypertension-specific therapy could be definitively recommended. They recommended that treatment decisions be made jointly with a sarcoidosis and pulmonary hypertension expert. The committee recognised that there were significant defects in the current knowledge regarding SAPH, but felt the statement would be useful in directing future studies.
结节病相关性肺动脉高压(SAPH)是晚期结节病的重要并发症。在过去的几年中,已经有几项研究涉及 SAPH 的筛查、诊断和治疗。这包括两个大型 SAPH 特异性注册研究的结果。世界结节病和其他肉芽肿性疾病协会(WASOG)成立了一个工作组,总结该领域的现有知识水平,并为患者的管理提供指导。一组结节病和肺动脉高压专家参与了这个工作组。委员会就初始筛查达成了共识,包括谁应该进行更具体的超声心动图检查。根据结果,委员会同意谁应该进行右心导管检查以及如何解释结果。委员会认为,没有哪种 SAPH 患者具有特定的表型,可以明确推荐使用肺动脉高压特异性治疗。他们建议与结节病和肺动脉高压专家共同做出治疗决策。委员会认识到,目前对 SAPH 的认识存在重大缺陷,但认为该声明将有助于指导未来的研究。