Pulmonary Hypertension in Patients with Chronic Fibrosing Idiopathic Interstitial Pneumonias.
- Hoeper MM Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany.
- Behr J Department of Internal Medicine V, University of Munich, Munich, Germany.
- Held M Department of Internal Medicine, Respiratory Medicine and Cardiology, Mission Medical Hospital, Würzburg, Germany.
- Grunig E University Hospital Heidelberg, Heidelberg, Germany.
- Vizza CD Department of Cardiovascular and Respiratory Diseases, Sapienza, University of Rome, Rome, Italy.
- Vonk-Noordegraaf A Department of Pulmonary Diseases, VU University Medical Center, Amsterdam, The Netherlands.
- Lange TJ Department of Internal Medicine II, Division of Pneumology, University Medical Center Regensburg, Regensburg, Germany.
- Claussen M LungenClinic, Grosshansdorf, Germany.
- Grohé C Department of Respiratory Medicine, ELK Thorax Centre, Berlin, Germany.
- Klose H University Medical Center Hamburg-Eppendorf, Center of Oncology, Department of Respiratory Medicine, Hamburg, Germany.
- Olsson KM Department of Respiratory Medicine and German Center of Lung Research (DZL), Hannover Medical School, Hannover, Germany.
- Zelniker T Department of Cardiology, Angiology and Pneumology, University of Heidelberg, Heidelberg, Germany.
- Neurohr C Department of Internal Medicine V, University of Munich, Munich, Germany.
- Distler O Division of Rheumatology, University Hospital Zurich, Zurich, Switzerland.
- Wirtz H Department of Respiratory Medicine, University of Leipzig, Leipzig, Germany.
- Opitz C Department of Cardiology, DRK Kliniken Berlin Köpenick, Berlin, Germany.
- Huscher D Department of Rheumatology and Clinical Immunology, Charité University Hospital, and Epidemiology unit, German Rheumatism Research Centre, Berlin, Germany.
- Pittrow D Institute for Clinical Pharmacology, Medical Faculty, Technical University, Dresden, Germany.
- Gibbs JS Department of Cardiology, National Heart & Lung Institute; Imperial College London, London, United Kingdom.
- 2015-12-03
Published in:
- PloS one. - 2015
Aged
Chronic Disease
Female
Humans
Hypertension, Pulmonary
Idiopathic Interstitial Pneumonias
Male
Middle Aged
Prognosis
Prospective Studies
Pulmonary Fibrosis
Registries
Survival Rate
Vasodilator Agents
English
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.
TRIAL REGISTRATION
clinicaltrials.gov NCT01347216.
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.
TRIAL REGISTRATION
clinicaltrials.gov NCT01347216.
- Language
-
- English
- Open access status
- gold
- Identifiers
-
- DOI 10.1371/journal.pone.0141911
- PMID 26630396
- Persistent URL
- https://sonar.ch/global/documents/66222
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