Polymyositis/dermatomyositis/clinically amyopathic dermatomyositis-associated Interstitial lung disease
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: The diagnosis of PM or DM was based on the criteria of Bohan and Peter criteria: 1) systemic muscle weakness, 2) increased serum muscle enzyme levels, 3) electromyographic (EMG) evidence of myopathic changes, 4) typical histologic findings in muscle biopsies, and/or 5) characteristic dermatologic manifestations of DM. The diagnosis was considered definite, probable, or possible according to the number of criteria fulfilled (at least 4, 3, or 2, respectively, including the dermatologic manifestations for diagnosis of DM), and patients with definite or probable PM/DM were included in the study. CADM was diagnosed when a patient had a skin rash characteristic of DM without clinical evidence of muscle disease and with little or no increase in the serum creatine kinase (CK) level. Interstitial lung disease was diagnosed on the basis of the presence of high resolution computed tomography abnormalities in combination with one or more of the following; dyspnea on exertion, serum KL-6 level > 500 U/ml, arterial oxygen tension (PaO2) < 80mmHg, %FVC < 80%, %DLCO < 65%.
Exclusion criteria
Exclusion criteria: Patients who are unable to get informed consent
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Clinical feature and overall survival according to MSA status | — |
Secondary
| Measure | Time frame |
|---|---|
| Prevalence of each myositis-specific autoantibodies Change of clinical symptoms, pulmonary function tests, chest HRCT findings, and laboratory findings during follow up period (3 years) Comparison of therapeutic regimen | — |
Countries
Japan
Contacts
Hamamatsu University School of Medicine Second Division, Department of Internal Medicine