polymyositis/dermatomyositis/clinically amyopathic dermatomyositis-associated Interstitial lung disease
Conditions
Interventions
arm1: tacrolimus and predonisolon therapy for 52 weeks Initial dose of oral prednisolone is 0.6 - 1 mg/kg/day. Intravenous methylprednisolone pulse therapy (1 g/day for 3 days) is permitted accord
Sponsors
Second Division, Department of Internal medicine, Hamamatsu University School of Medicine
Eligibility
Sex/Gender
All
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 met the following criteria are excluded from this study: (i) no evidence of deterioration in interstitial lung disease for more than 6 months; (ii) the presence of a serious comorbidity (e.g. malignancy, liver dysfunction, and renal dysfunction); (iii) use of immunosuppressants (except for corticosteroid), intravenous immunoglobulin therapy, plasma exchange.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Progression free survival rate at week 52 | — |
Secondary
| Measure | Time frame |
|---|---|
| Overall survival rate at week 52 Change in forced vital capacity from baseline at week 52. Change in DLCO, 6MWT,PaO2, KL-6 and SP-D from baseline at week 4, 12, 24 and 52. | — |
Countries
Japan
Contacts
Public ContactTomoyuki Fujisawa
Hamamatsu University School of Medicine Second Division, Department of Internal Medicine
Outcome results
None listed