Progress in the Treatment of Pulmonary Hypertension Associated with Interstitial Lung Disease

Pulmonary hypertension often complicates fibrotic interstitial lung disease, leading to higher morbidity and mortality. Medications designed for pulmonary arterial hypertension are now used for interstitial lung disease patients with pulmonary hypertension, though uncertainty remains whether treatment is beneficial. Studies have shown conflicting results, some indicating benefits while others harm. Recent advancements include the first approved therapy in the US for interstitial lung disease with pulmonary hypertension, marking a paradigm shift. The review offers insights into past studies, challenges in drug development, and a practical management approach considering changing definitions, comorbid factors, and emerging treatment options.