Idiopathic Pleuroparenchymal Fibroelastosis (PPFE)
Conditions
Interventions
None listed
Sponsors
Kindai University
Kitano Hospital, The Tazuke Kofukai Medical Research Institute Kobe City Medical Center General Hospital Tosei General Hospital Kanagawa Cardiovascular and Respiratory Center Nagasaki University Hospital Shiga University of Medical Science Hospital Kyoto University National Hospital Organization Minami Kyoto Hospital National Hospital Organization Kyoto Medical Center Kyoto Katsura Hospital Kindai University Nara Hospital Tenri Hospital Japanese Red Cross Taka
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1.Patients diagnosed with idiopathic pleuroparenchymal fibroelastosis (iPPFE), chronic obstructive pulmonary disease (COPD), or idiopathic pulmonary fibrosis (IPF), whose clinical condition has been stable for at least 4 weeks, or healthy adults. 2.Patients with a modified Medical Research Council (mMRC) dyspnea score >1. 3.Written informed consent obtained prior to participation.
Exclusion criteria
Exclusion criteria: 1.Pneumothorax within 3 months before enrollment. 2.Receiving long term oxygen therapy. 3.Musculoskeletal disorders affecting upper or lower limb muscle strength. 4.Connective tissue diseases interfering with physical function assessment. 5.Cerebrovascular disease with clinically significant neurological deficits. 6.6. Ongoing pulmonary rehabilitation at enrollment (completion at least 6 months before enrollment is allowed). 7.Any condition judged by the investigator to make participation inappropriate.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Difference in exercise endurance time between sitting-cycle ergometer testing and supine-cycle ergometer testing in patients with idiopathic pleuroparenchymal fibroelastosis (PPFE) | — |
Secondary
| Measure | Time frame |
|---|---|
| 1.Factors associated with the difference in exercise endurance time between sitting and supine positions in patients with PPFE. 2.Differences in ventilatory efficiency between sitting and supine positions and factors associated with these differences in patients with PPFE. 3.Comparisons of exercise endurance time, ventilatory efficiency, and diaphragmatic excursion among patients with PPFE, COPD, IPF, and healthy controls. 4.Changes in 6-minute walk distance, exercise endurance time, and ventilatory efficiency after pulmonary rehabilitation and factors associated with these changes. | — |
Countries
Japan
Contacts
Public ContactMasashi Shiraishi
Kindai University Hospital Department of Rehabilitation
Outcome results
None listed