anti-aminoacyl-tRNA synthetase antibody-positive polymyositis/dermatomyositis/clinically amyopathic dermatomyositis-associated interstitial lung disease
Conditions
Interventions
Arm 1: corticosteroid (prednisolone) monotherapy for 24 months Initial dose of oral prednisolone is 0.7 - 1mg/kg/day. (Maximum dose of prednisolone is 60mg/body/day.) Intravenous methylprednisolon
Sponsors
Hamamatsu University School of Medicine
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: PM/DM/CADM-ILD patients who are positive for any of serum anti-aminoacyl tRNA-synthetase antibodies including anti-Jo-1 antibody and have not previously received treatment for PM/DM/CADM-ILD are included in this study. Bohan and Peter criteria with slight modification: 1) systemic muscle weakness or myalgia, 2) increased serum muscle enzyme levels, 3) electromyographic (EMG) evidence of myopathic changes or muscular signal abnormalities on MRI, 4) typical histologic findings in muscle biopsies, and/or 5) characteristic dermatologic manifestations of DM. The diagnosis is 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 are included in the study. Sontheimer criteria with slight modification: CADM is diagnosed when a patient had 1) a skin rash characteristic of DM 2) without clinical evidence of muscle disease and 3) with little or no increase in the serum creatine kinase (CK) level during the observation period. ILD: ILD is diagnosed on the basis of the presence of high resolution computed tomography (HRCT) abnormalities in combination with one or more of the following; 1) progressive on HRCT, 2) dyspnea on exertion (modified MRC score 1 or more), 3) PaO2 < 80 Torr, 4) %FVC < 80% or %DLCO < 80%.
Exclusion criteria
Exclusion criteria: Patients who meet the following criteria are excluded from this study: (1) Patients who requires systemic high dose corticosteroid, immunosuppressants, intravenous immunoglobulin therapy, plasma exchange, or biologic agents for a disease other than PM/DM/CADM-ILD at the registration (2) Patients with severe respiratory failure (PaO2 < 50 Torr) (3) Patients with contraindication of prednisolone or tacrolimus (4) Patients with a serious comorbidity (e.g. advanced malignancy, imminent aortic aneurysm, and Liver cirrhosis) (5) Patients with anti-MDA5 antibody (6) Patients who are judged unqualified for this study by attending physician
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Progression free survival and progression free survival rate at 12 and 24 month | — |
Secondary
| Measure | Time frame |
|---|---|
| Disease control rate at 1 month Recurrence free survival and recurrence free survival rate at 12 and 24 month Incidence of adverse events Overall survival and overall survival rate at 12 and 24 month Non-elective hospitalization rate (all-cause, PM/DM/CADM-ILD related, and non-PM/DM/CADM-ILD related) Change in PM/DM/CADM-ILD related symptom, FVC, FEV1, KL-6, SP-D, anti-aminoacyl-tRNA synthetase antibody titer, Chest HRCT findings) | — |
Countries
Japan
Contacts
Public ContactHironao Hozumi
Hamamatsu University School of Medicine Second Division, Department of Internal Medicine
Outcome results
None listed