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.
* 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.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
TREATMENT
Masking
NONE
Enrollment
15
Pulmonary Medicine, Asan Medical Center, Ulsan University
Seoul, South Korea
Safety
Pulmonary arterial pressure
Exercise capacity (6 minute walking test)
6 minute walking test: Min. oxygen saturation.
NYHA class,
Quality of life (St. George Respiratory questionnaires)
Pulmonary vascular resistance, cardiac output.
Increment of pulmonary arterial pressure after the exercise
6) Pulmonary function test
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