Efficacy and Safety of Pirfenidone in Patient With Dermatomyositis Interstitial Lung Disease (Dm-ILD)

Inclusion Criteria:

1. Female or male subjects aged between 18 and 65 years of age

2. According to the 1975 Borhan and Peter inflammatory myopathy diagnosed asdermatomyositis classification criteria (dermatomyositis, DM); or according to therevised clinical criteria for diagnosis of Sontheimer disease dermatomyositis (CADM).

3. The diagnosis of pulmonary related Interstitial Lung Disease confirmed by HRCT

4. Forced vital capacity (FVC) 40% to 80% predicted(include 40% and80% )

5. Carbon monoxide diffusing capacity (DLco) 30% to 89% of predicted normal(include 30%and89% )

6. Has received Glucocorticoid (hereinafter referred to as the "hormone") and at leastone immunosuppressive therapy for more than 3 months, the hormone dosage (prednisoneequivalent dose calculation) should be less than 15mg/d for at least 1 months, shouldbe immunosuppressant cyclophosphamide, mycophenolate mofetil, cyclosporine,tacrolimus, azathioprine and at least one of methotrexate in, and the type and dose ofimmunosuppressive agents should be at least stable more than 3 months. The hormone andimmunosuppressant therapy scheme allows a reduction in the study period, but is notallowed to increase the volume.

7. Women of childbearing age must agree and promise to use the form of medical care forat least 3 months after the trial, during the entire study period (includingfollow-up), and at the end of the trial.

8. Patients volunteered to participate in the trial, with good compliance and ability tounderstand and sign informed consent before the study.

Exclusion Criteria:

1. Subjects not fulfill all of the above inclusion criteria

2. Combined with other rheumatic diseases such as systemic sclerosis, systemic lupuserythematosus, Sjogren's syndrome, mixed connective tissue disease, undifferentiatedconnective tissue disease, and systemic vasculitis, such as ANCA associatedvasculitis.

3. Combined with other muscle diseases and may cause symptoms of myasthenia gravisdisease, including neurological diseases (such as muscular dystrophy, myastheniagravis, amyotrophic lateral sclerosis, Guillain Barre syndrome), cancer, drugs (suchas statins), infection, genetic diseases, endocrine disorders, electrolyte disorderrhabdomyolysis.

4. The clinical history, signs, serological examination, HRCT and bronchoalveolar lavageresults suggest that in addition to inflammatory myopathy and other diseases caused byILD, such as CTD, systemic vasculitis, infection, tumor, allergic pneumonia caused bysarcoidosis or environmental factors.

5. Combined viscera function significantly abnormal patient:

6. Liver:AST, ALT >1.3ULN;Bilirubin >1.5 ULN; Cirrhosis of the liver class for ChildPugh C;

7. Kidney:Creatinine clearance <30 mL/min;

8. Lung:Airway obstruction (pre-bronchodilator FEV1/FVC <0.7);Other clinicallysignificant pulmonary abnormalities;

9. Cardiovascular:i.Six weeks in severe hypertension, and out of control aftertreatment(≥160/100mmHg);ii.Myocardial infarction within six months;iii.A periodof 6 Months in unstable angina;iv.pulmonary artery hypertension and right heartfailure were significant;

10. Gastrointestinal tract: with active peptic ulcer;

11. Nervous system: Patients with psychiatric disorders;

12. The blood coagulation function: History of thrombotic event within lastyear(Including stroke and transient ischemic attack).

13. Researchers, for other diseases (not inflammatory myopathy, such as malignant tumor)and make the life expectancy of < 1 year of patients

14. Allergic to test drugs or components (e.g. lactose)

15. Patients with actinic dermatitis

16. Previous treatment with nintedanib or pirfenidone

17. Within 3 months to participate in other clinical trials;

18. Combined medication: hormone > 15mg/d, were less than 1 months before a stable dose ofpatients; the use of other immunosuppressive agents (except cyclophosphamide,mycophenolate mofetil, cyclosporine, tacrolimus, azathioprine, methotrexate and)patients; immunosuppression stable < 3 months; during the study of hormones andimmunosuppressive agents are likely to increase patients.

19. Major surgery is planned during the treatment period.

20. Pregnancy or lactation or make a schedule during the trials.

21. Give the drug 28 days before or after administration of the 3 month period, women ofchildbearing age * are unwilling or unable to use contraceptive methods highlyeffective (according to ICH M3 (R2)), a highly effective means in the correct andconsistent application of a barrier method when the failure rate of less than 1% peryear. * women of childbearing age is defined has undergone menarche and in line with "infertile women" standard "[female infertile women" is defined as: postmenopausalperiod (12 months without menstruation, no other medical reasons) or permanentsterilization (e.g., tubal occlusion, hysterectomy, bilateral ovarian resection orbilateral tubal resection women)].

22. According to the researchers,exhibited evidence of alcohol or drug abuse.

23. Patients who were unable to understand or comply with the study procedures, includingthe completion of a self-administered questionnaire in the absence of help, were lesslikely to complete the trial.

24. Severe limb weakness or joint disease that affects the stability and endurance of apatient who is unable to perform a 6 minute walking test.

25. Clinical signs of malabsorption or needing parenteral nutrition.

26. Patients who were unable to cope with pulmonary function tests.

27. With mental illness .

28. Researchers determined that they did not participate in the trial.