Polymyositis/dermatomyositis complicated with interstitial lung disease (PM/DM-ILD)
Conditions
Interventions
None listed
Sponsors
Nagasaki University
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) Patients diagnosed with PM/DM according to the criteria of the Ministry of Health, Labour and Welfare, Bohen and Peter criteria, or Sontheimer's Clinically amyopathic dermatomyositis (CADM), or patients with positive myositis-specific autoantibodies 2) Patients with ILD 3) Patients who are 20 years of age or older at the time of obtaining consent. 4) Patients planning to receive immunosuppressive therapy or receiving corticosteroids only within 8 weeks. 5) Patients with fibrotic lung disease that affects lung capacity by more than 10% on high-resolution CT. 6) Patients with FVC more than 50% of predicted and DLCO of 20-89% predicted. 7) ability and willingness to provide written informed consent and comply with the requirements of the study protocol.
Exclusion criteria
Exclusion criteria: 1) Patients who have received oral corticosteroids for more than 8 weeks prior to obtaining consent. 2) Patients with malignant tumors. 3) Patients who are using or have used an anti-fibrotic therapy. 4) Patients who have undergone more than lobectomy. 5) Other patients deemed inappropriate by the investigator.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Search for biomarkers associated with change in high-resolution CT fibrosis volume and FVC change at 52 weeks from baseline | — |
Secondary
| Measure | Time frame |
|---|---|
| FVC at baseline assessment, 26 weeks, and 52 weeks 5% or more deterioration in relative change in %FVC at 52 weeks 5% or more improvement in relative change in %FVC at 52 weeks 10% or more deterioration in relative change in %FVC at 52 weeks 10% or more improvement in relative change in %FVC at 52 weeks Lung fibrosis score at baseline assessment and at 52 weeks | — |
Countries
Japan
Contacts
Public ContactTakashi Igawa
Nagasaki University Graduate School of Biomedical Sciences Department of Immunology and Rheumatology
Outcome results
None listed