Pulmonary Hypertension in Interstitial Lung Disease Diagnosis and Management

In Brief The association of pulmonary hypertension (PH) and interstitial lung disease (ILD) is well known. However, the etiology is likely multifactorial and the prevalence varied. When PH presents in a patient with ILD, it is associated with increased morbidity and mortality. Noninvasive diagnostic tools for PH in advanced lung disease have emerged but definitive diagnosis requires right heart catheterization. No guidelines currently exist for therapy in this secondary form of PH. Small case series and studies have shown mixed results in the use of therapy for Group I pulmonary arterial hypertension in secondary PH. Further studies are needed to define the role of these therapies in PH of ILD. Pulmonary hypertension is commonly found in association with interstitial lung disease. This finding heralds increased mortality and increased symptoms but also presents potential targets for therapy.