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Abstract

<jats:p>Pulmonary hypertension (PH) is a progressive and debilitating condition that commonly complicates interstitial lung disease (ILD), forming a clinical entity known as PH-ILD, categorized under Group 3 PH by ESC/ERS guidelines. Its pathogenesis is multifactorial, involving chronic alveolar hypoxemia, hypoxic vasoconstriction, vascular remodeling, and inflammatory mediators, ultimately leading to increased pulmonary vascular resistance and right heart strain. Recent advancements in diagnostic strategies—including echocardiography, CT scan and biomarkers such as NT-proBNP—have improved early recognition of PH-ILD. NT-proBNP levels below 95 ng/L can help rule out PH with up to 99% predictive value. Moreover, pulmonary function testing, particularly DLCO, offers valuable diagnostic and prognostic information, serving as a mortality predictor in PH-ILD, unlike standard spirometry. Although past studies suggested limited benefit of pulmonary vasodilators in PH-ILD, newer evidence has reshaped clinical perspectives. The INCREASE trial demonstrated that inhaled treprostinil significantly improved exercise tolerance and pulmonary hemodynamics in PH-ILD patients. Additionally, data from the COMPERA registry and trials like STEP-IPF provide insight into patient stratification and response to therapy. This review summarizes current knowledge on the pathophysiology, diagnosis, and treatment of PH-ILD, emphasizing the value of non-invasive tools and emerging therapies. These advancements offer clinicians practical frameworks to improve outcomes and quality of life for patients with this challenging condition.</jats:p>

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Keywords

phild pulmonary condition clinical vascular

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