Idiopathic interstitial pneumonia-associated pulmonary hypertension: A target for therapy?

- IIPs involve varying combinations of fibrosis and inflammation of unknown cause.
- IIPs appear to follow a similar course once PH supervenes.
- Exploring PH drugs in broadly defined IIP, rather than exploring only specific subtypes, such as IPF, may be of benefit.
- RISE-IIP looked to validate riociguat in patient with IIP-PH in a randomized trial.

Advances in the treatment of idiopathic interstitial pneumonia (IIP) represent an urgent, unmet medical need for patients with this category of diffuse parenchymal lung disease. IIPs involve varying combinations of fibrosis and inflammation of unknown cause and may be associated with pulmonary hypertension (PH). When it occurs, PH is associated with higher oxygen needs, greater functional impairment, and increased mortality. However, whether or when PH is a maladaptive versus adaptive phenomenon remains to be determined. Despite their differing prognoses, it does appear that the IIPs may follow a similar course once PH supervenes. Therefore, it may be worthwhile to explore studies of PH medications in IIP as a group rather than as individual entities. Such a broad approach eliminates the need to nuance specific diagnoses and thereby facilitates study recruitment and broadens the applicability of the results.