Kacprzak Aneta, Tomkowski Witold, Szturmowicz Monika
1st Department of Lung Diseases, National Tuberculosis and Lung Diseases Institute, Plocka 26, 01-138 Warsaw, Poland.
Diagnostics (Basel). 2023 Jul 13;13(14):2354. doi: 10.3390/diagnostics13142354.
The prevalence of pulmonary hypertension (PH) complicating interstitial lung diseases (ILDs) is 3.5-15% at an early stage, and up to 90% in ILD patients listed for lung transplantation. In addition, other types of PH may occur in patients with ILDs due to concomitant conditions. Therefore, any significant PH occurring in the setting of ILD requires a proper differential workup. PH increases morbidity and mortality in ILDs. The pathomechanisms underlying PH due to ILD (PH-ILD) are not fully known, and there is no straightforward correlation between the presence or severity of PH-ILD and the severity of ILD. Severe PH in mild ILD without other explanatory causes constitutes a dilemma of differentiating between PH due to ILD and pulmonary arterial hypertension coexisting with ILDs. The heterogeneity and poor prognosis of patients with ILDs coexisting with PH necessitate an individualised approach to the management of this condition. This review presents recent advances in understanding and treatment options in PH-ILD. It also addresses practical issues, such as when to suspect and how to screen for PH in ILD, what are the indications for right heart catheterisation, and how to approach an individual ILD patient to determine the dominant PH cause and apply adequate management.
间质性肺疾病（ILD）合并肺动脉高压（PH）的患病率在疾病早期为3.5%-15%，在等待肺移植的ILD患者中高达90%。此外，由于并存疾病，ILD患者可能会出现其他类型的PH。因此，在ILD背景下出现的任何显著PH都需要进行适当的鉴别检查。PH会增加ILD患者的发病率和死亡率。ILD相关PH（PH-ILD）的发病机制尚未完全明确，PH-ILD的存在或严重程度与ILD的严重程度之间也没有直接关联。轻度ILD且无其他解释性原因的严重PH构成了鉴别ILD相关PH与与ILD并存的肺动脉高压的难题。ILD合并PH患者的异质性和不良预后需要针对这种情况采取个体化的管理方法。本综述介绍了PH-ILD在认识和治疗选择方面的最新进展。它还讨论了一些实际问题，例如何时怀疑以及如何在ILD中筛查PH，右心导管检查的指征是什么，以及如何针对个体ILD患者确定主要的PH病因并采取适当的管理措施。
