title: Idiopathic interstitial pneumonia-associated pulmonary hypertension: A target for therapy?
abstract: 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.
authors.affiliation: Inova Fairfax Hospital, Vienna, VA, USA. Electronic address: Steven.Nathan@inova.org.
authors.forename: Steven D
authors.initials: SD
authors.lastname: Nathan
authors.affiliation: Department of Internal Medicine V, University of Munich, Member of the German Center for Lung Research, Munich, Germany.
authors.forename: Jürgen
authors.initials: J
authors.lastname: Behr
authors.affiliation: National Reference Center for Rare Pulmonary Diseases, Regional Center for Pulmonary Hypertension, Louis Pradel Hospital, Hospices Civils de Lyon, Claude Bernard Lyon 1 University, Lyon, France.
authors.forename: Vincent
authors.initials: V
authors.lastname: Cottin
authors.affiliation: University of California San Francisco, San Francisco, CA, USA.
authors.forename: Harold R
authors.initials: HR
authors.lastname: Collard
authors.affiliation: Hannover Medical School and Hannover and German Centre for Lung Research, Hannover, Germany.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.affiliation: Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, Weill Cornell Medical School, New York, NY, USA.
authors.forename: Fernando J
authors.initials: FJ
authors.lastname: Martinez
authors.affiliation: Royal Brompton & Harefield, NHS Foundation, London, England, United Kingdom.
authors.forename: Athol U
authors.initials: AU
authors.lastname: Wells
mesh_terms: D049109:Cell Proliferation / Q000187:drug effects; D002986:Clinical Trials as Topic; D020536:Enzyme Activators / Q000627:therapeutic use; D005109:Extracellular Matrix / Q000187:drug effects; D005347:Fibroblasts / Q000187:drug effects / Q000473:pathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy; D054988:Idiopathic Interstitial Pneumonias / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D009569:Nitric Oxide / Q000378:metabolism; D011379:Prognosis; D011720:Pyrazoles / Q000378:metabolism / Q000494:pharmacology; D011743:Pyrimidines / Q000378:metabolism / Q000494:pharmacology; D014481:United States
chemical_list: D020536:Enzyme Activators; D011720:Pyrazoles; D011743:Pyrimidines; D009569:Nitric Oxide; C542595:riociguat
keywords: Idiopathic interstitial pneumonia; Idiopathic pulmonary fibrosis; Interstitial lung disease; Pulmonary hypertension