Pulmonary Hypertension in the Course of Interstitial Lung Diseases—A Personalised Approach Is Needed to Identify a Dominant Cause and Provide an Effective Therapy

Abstract

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.

Understanding pulmonary hypertension: the need for an integrative metabolomics and transcriptomics approach

“…This results in poor prognosis and worse survival outcomes. 80 A total of three studies indicate altered gene expression in PH patients with different ILD subtypes such as IPF 81,82 and sarcoidosis. 83 Extracellular matrix remodelling was found to be majorly involved in these cases.…”