Idiopathic Pulmonary Fibrosis
Conditions
Interventions
Sponsors
Nippon Medical School
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) Patients with IPF enrolled under UMIN ID: UMIN000042159 who completed the two year observation period. 2) Patients aged 20 years old or older (both sexes) 3) Patients diagnosed at this hospital as having IPF (WHO functional class 2, 3 or 4) without hypoxia at rest or during 6MWT (to exclude those with decreased ADL and dyspnea in daily living associated with hypoxia and to minimize the influence of hypoxic pulmonary vasoconstriction [HPV] as a potential cause of PH associated with decreased partial pressure of oxygen in arterial blood [PaO2]) (PaO2 < 60 mmHg). Including those whose hypoxia (PaO2 < 60 mmHg) had been corrected with long-term oxygen therapy (LTOT) 4) Patients with stable IPF who had not required any change of treatment within 3 months prior to study entry, i.e., those confirmed to have completely organized honeycomb lung (chronic IIP based on high-resolution computed tomography (CT) findings for which no effective therapy exists); and who presented to our hospital for the first time with symptoms of progressive respiratory failure and had not received any medical treatment for IPF within 3 months prior to their visit. Excluding those whose progressive respiratory failure required no treatment for IPF itself and those who had an increased LTOT dose as a minimum requirement for progressive respiratory failure. 5) Patients with eePAP or PH requiring close clinical monitoring, diagnosed with an assumed PAWP 15 mmHg and less than 15 mmHg, mPAP < 25 mmHg, and mPAP during the Valsalva maneuver 30 mmHg and more than 30 mmHg, or mPAP at rest 25 mmHg and more than 25 mmHg. 6) Inpatients and outpatients 7) Patients who provided written informed consent to participate in this study
Exclusion criteria
Exclusion criteria: 1) Patients who had received bosentan or any other drug specific for PAH (e.g., phosphodiestetrase type 5 [PDE-5] inhibitors, endothelin receptor antagonists, or prostaglandin analogs) prior to their enrollment 2) Patients with any disease that could cause right heart overload 3) Patients with hypoxia during 6MWT (PaO2 < 60 mmHg)*. * Excluded were those whose hypoxia (PaO2 < 60 mmHg) had been corrected with LTOT (i.e., those in whom LTOT is in place to ensure PaO2 > 60 mmHg both at rest and during 6MWT, who were deemed equivalent to IPF patients receiving routine therapy in clinical practice to allow them to be monitored for changes in their condition, prognosis and functional capacity for ADL). 4) Women who were pregnant or might have been pregnant, and who were lactating 5) Other patients judged by the investigator to be ineligible for this study (e.g., those with any disease or condition other than IPF that might affect their ADL, such as arrhythmia, LV failure, pulmonary thromboembolism, connective tissue diseases, intervertebral disc herniation, as they were confirmed by history taking, physical examination, chest x-ray, echocardiography [ECG], lung perfusion scintigraphy, and measurements of various parameters conducted during the run-in period).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| To examine the relationship between pulmonary circulation parameters, including pulmonary vascular resistance (PVR) and the prognosis in patients with idiopathic pulmonary fibrosis (IPF). | — |
Secondary
| Measure | Time frame |
|---|---|
| To examine the relationship between pulmonary circulation parameters, including pulmonary vascular resistance (PVR), and the activities of daily living (ADL) and exercise tolerance in patients with idiopathic pulmonary fibrosis (IPF). | — |
Countries
Japan
Contacts
Public ContactYosuke Tanaka
Nippon Medical School Department of Respiratory Medicine
Outcome results
None listed