Pulmonary Hypertension in Idiopathic Pulmonary Fibrosis: A Review

Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal disease with a poor prognosis. Pulmonary hypertension (PH) often complicates the course of IPF and may even be found in patients with preserved lung function. Possible pathogenetic mechanisms of PH in IPF include vascular destruction, pulmonary hypoxic vasoconstriction and vascular remodeling due to overexpression of cytokines and growth factors. PH in IPF patients is associated with decreased exercise capacity and a worse prognosis. Due to its prognostic significance, it seems important to investigate for PH in these patients. As the symptoms of PH in IPF are nonspecific, the development of PH in a patient with known IPF can be easily overlooked. Noninvasive methods provide clues for the diagnosis, but their sensitivity is limited. Doppler echocardiography is a useful tool for the detection of PH which also provides additional information regarding associated cardiac abnormalities. However, right heart catheterization remains the gold standard diagnostic test. Therapeutic options for PH in IPF are limited. Long-term oxygen administration for the correction of hypoxemia should be recommended. The availability of new pharmacological agents in the treatment of PH has raised the possibility of therapy in patients with IPF and associated PH. Whether these PH-targeted therapies may be of benefit in this patient group, in terms of improving functional outcomes and survival, remains uncertain.