Pulmonary Fibrosis, Pulmonary Hypertension
Conditions
Keywords
Pulmonary fibrosis, pulmonary hypertension, Iloprost, Efficacy
Brief summary
Idiopathic pulmonary fibrosis(IPF) is chronic progressive fibrosing lung disease of unknown cause. There is no effective therapy yet for this disease and the mean survival in most reports is about 3 years after the diagnosis. Because of the stiff fibrosis of the lung, pulmonary hypertension is the late complication of IPF and its development heralds a very poor outcome of the patients. For the primary pulmonary hypertension, recently the effective drugs have been available. However, there is no study about the efficacy of these drugs in the patients with pulmonary hypertension secondary to pulmnary fibrosis, and the aim of this trial is to study the safty and efficacy of Iloprost, one of the safe and effective drugs in primary pulmonary hypertension.
Detailed description
* Prospective open labeled observational study * Subjects: About 15 patients with secondary pulmonary hypertension due to IPF or pulmonary fibrosis associated with collagen vascular diseases. * Method: 3 month trial of inhaled iloprost. Check the safty and measure the pulmonary arterial pressure by right heart catheterization, exercise capacity by 6 minute walking test, echocardiography, and quality of life questionnaires before and after the trial.
Interventions
Sponsors
Interstitial Lung Disease Study Group, Korea
Study design
Allocation
NON_RANDOMIZED
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Diagnosis of idiopathic pulmonary fibrosis or fibrotic NSIP according to American Thoracic Society and European Respiratory Society guidelines by biopsy and diagnosis of pulmonary fibrosis associated with connective tissue disease. * Mean pulmonary artery pressure over 30mmHg. * NYHA functional class II to IV
Exclusion criteria
* Suffering lung diseases other than pulmonary fibrosis (COPD, Pulmonary Thromboendarterectomy ). * Administration of prostanoids, bosentan, beta- blocker or phosphodiesterase5 inhibitor. * Dosage adjustment of calcium channel blockers within 6 weeks. * Resting pulmonary capillary wedge pressure over 15mmHg. * Bleeding tendency. * Bilirubin level above 3mg/dl or creatinine clearance level below 30ml/min. * Unstable angina pectoris, myocardial infarction or severe arrhythmia within 6 months. * Cerebrovascular accident within 6 months. * Present lung infection.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Safety | — |
| Pulmonary arterial pressure | — |
| Exercise capacity (6 minute walking test) | — |
Secondary
| Measure | Time frame |
|---|---|
| Pulmonary vascular resistance, cardiac output. | — |
| 6 minute walking test: Min. oxygen saturation. | — |
| 6) Pulmonary function test | — |
| Increment of pulmonary arterial pressure after the exercise | — |
| NYHA class, | — |
| Quality of life (St. George Respiratory questionnaires) | — |
Countries
South Korea
Contacts
Primary ContactDong Soon Kim, MD
Backup ContactSook Hee Jung, RN
Outcome results
None listed