When interstitial lung diseases associates pulmonary hypertension, what to do?

Pulmonary hypertension (PH) is one of the major comorbidities of interstitial lung diseases (ILD) and negatively impacts on patients’ symptoms and prognosis. PH-ILD diagnosis is made following a three-step strategy. In a first step, suspicion of PH-ILD should be raised if a combination of symptoms, signs, functional impairments, radiological features, and biomarkers is present. In a second step, patients in whom PH-ILD is suspected should undergo transthoracic echocardiography (TTE) for PH screening. In a third step, depending on TTE findings and clinical suspicion, right heart catheterization should be performed to diagnose or rule out PH. Once PH-ILD is diagnosed, a holistic approach is needed, considering optimization of the underlying ILD, assessment of comorbidities, pulmonary rehabilitation, oxygen supplementation, symptom control, evaluation for lung transplantation, and PH therapy. The decision about PH-targeted therapies should be made using a multimodal approach in a multidisciplinary PH-ILD committee.