Science and Research

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

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.

  • Nathan, S. D.; Behr, J.; Cottin, V.; Collard, H. R.; Hoeper, M. M.; Martinez, F. J.; Wells, A. U.

Keywords

  • Idiopathic interstitial pneumonia
  • Idiopathic pulmonary fibrosis
  • Interstitial lung disease
  • Pulmonary hypertension
Publication details
DOI: 10.1016/j.rmed.2016.11.003
Journal: Respiratory medicine
Pages: S10-S13 
Work Type: Review
Location: BREATH
Disease Area: PH
Partner / Member: MHH
Access-Number: 27884593
See publication on PubMed

DZL Engagements

chevron-down