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Research Article: Latin American expert opinion paper on the diagnosis and treatment of pulmonary hypertension associated with interstitial lung disease

Date Published: 2026-05-26

Abstract:
Pulmonary hypertension associated with interstitial lung disease (PH-ILD) represents a major clinical challenge due to its impact on prognosis and the complexity of diagnosis and management. The objective of this work was to develop practical, region-specific guidelines primarily aimed at supporting healthcare professionals in Latin America. A multidisciplinary working group of Latin American experts in PH-ILD developed an expert opinion paper, from which a diagnostic and therapeutic algorithm was derived. The resulting algorithm provides a stepwise approach to identifying suspected PH in patients with interstitial lung disease, confirming the diagnosis, and guiding management and treatment. Clinical, functional, biological, and imaging findings, including transthoracic Doppler echocardiography, are integrated to estimate the probability of PH, with tricuspid regurgitation velocity used to stratify risk. Right heart catheterization is proposed to confirm the diagnosis and to inform treatment decisions according to current recommendations. This algorithm offers a structured and practical tool that may facilitate earlier diagnosis and support appropriate management of patients with PH-ILD in routine clinical practice.

Introduction:
Interstitial lung disease (ILD) includes a large group of more than 200 parenchymal lung disorders, most of which are classified as rare diseases. Treatment options vary depending on the aetiology, so it is important to properly define its classification ( 1–5 ). Similar to idiopathic pulmonary fibrosis (IPF), several other ILDs (such as hypersensitivity pneumonitis, ILD associated with autoimmune diseases, or unclassifiable idiopathic interstitial pneumonia [IIP]) may present with a progressive fibrosing…

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