title: Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias.
abstract: BACKGROUND
  Pulmonary hypertension (PH) is a common finding in patients with chronic fibrosing idiopathic interstitial pneumonias (IIP). Little is known about the response to pulmonary vasodilator therapy in this patient population. COMPERA is an international registry that prospectively captures data from patients with various forms of PH receiving pulmonary vasodilator therapies.
  
  
  METHODS
  We retrieved data from COMPERA to compare patient characteristics, treatment patterns, response to therapy and survival in newly diagnosed patients with idiopathic pulmonary arterial hypertension (IPAH) and PH associated with IIP (PH-IIP).
  
  
  RESULTS
  Compared to patients with IPAH (n = 798), patients with PH-IIP (n = 151) were older and predominantly males. Patients with PH-IIP were treated predominantly with phosphodiesterase-5 inhibitors (88% at entry, 87% after 1 year). From baseline to the first follow-up visit, the median improvement in 6MWD was 30 m in patients with IPAH and 24.5 m in patients with PH-IIP (p = 0.457 for the difference between both groups). Improvements in NYHA functional class were observed in 22.4% and 29.5% of these patients, respectively (p = 0.179 for the difference between both groups). Survival rates were significantly worse in PH-IIP than in IPAH (3-year survival 34.0 versus 68.6%; p<0.001). Total lung capacity, NYHA class IV, and mixed-venous oxygen saturation were independent predictors of survival in patients with PH-IIP.
  
  
  CONCLUSIONS
  Patients with PH-IIP have a dismal prognosis. Our results suggest that pulmonary vasodilator therapy may be associated with short-term functional improvement in some of these patients but it is unclear whether this treatment affects survival.
  
  
  BACKGROUND
  clinicaltrials.gov NCT01347216.
authors.affiliation: Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany.
authors.forename: Marius M
authors.initials: MM
authors.lastname: Hoeper
authors.affiliation: Department of Internal Medicine V, University of Munich, Munich, Germany.
authors.forename: Juergen
authors.initials: J
authors.lastname: Behr
authors.affiliation: Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany.
authors.forename: Matthias
authors.initials: M
authors.lastname: Held
authors.affiliation: University Hospital Heidelberg, Heidelberg, Germany.
authors.forename: Ekkehard
authors.initials: E
authors.lastname: Grunig
authors.affiliation: Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy.
authors.forename: C Dario
authors.initials: CD
authors.lastname: Vizza
authors.affiliation: Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
authors.forename: Anton
authors.initials: A
authors.lastname: Vonk-Noordegraaf
authors.affiliation: Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany.
authors.forename: Tobias J
authors.initials: TJ
authors.lastname: Lange
authors.affiliation: LungenClinic, Grosshansdorf, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Claussen
authors.affiliation: Department of Respiratory Medicine, ELK Thorax Centre, Berlin, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Grohé
authors.affiliation: University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany.
authors.forename: Hans
authors.initials: H
authors.lastname: Klose
authors.affiliation: Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany.
authors.forename: Karen M
authors.initials: KM
authors.lastname: Olsson
authors.affiliation: Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany.
authors.forename: Thomas
authors.initials: T
authors.lastname: Zelniker
authors.affiliation: Department of Internal Medicine V, University of Munich, Munich, Germany.
authors.forename: Claus
authors.initials: C
authors.lastname: Neurohr
authors.affiliation: Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.affiliation: Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany.
authors.forename: Hubert
authors.initials: H
authors.lastname: Wirtz
authors.affiliation: Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Opitz
authors.affiliation: Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany.
authors.forename: Doerte
authors.initials: D
authors.lastname: Huscher
authors.affiliation: Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany.
authors.forename: David
authors.initials: D
authors.lastname: Pittrow
authors.affiliation: Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom.
authors.forename: J Simon R
authors.initials: JS
authors.lastname: Gibbs
mesh_terms: D000368:Aged; D002908:Chronic Disease; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology* / Q000401:mortality / Q000473:pathology; D054988:Idiopathic Interstitial Pneumonias / Q000150:complications* / Q000401:mortality / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D011658:Pulmonary Fibrosis / Q000503:physiopathology*; D012042:Registries; D015996:Survival Rate; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents