A prospective cohort study was used to observe the efficacy and safety of different immunosuppressive agents with/wo pirfenidone on CTD-ILD patients in Qilu Hospital of Shandong University for 36 months.The main research endpoints are lung function, patient dyspnea score, 6-minute walking distance, imaging indicators, primary disease activity, adverse reactions, etc.

This study will enroll 200 cases of connective tissue disease-related interstitial lung disease (CTD-ILD) patients in China, including inflammatory myopathy (IIM) patients, rheumatoid arthritis (RA) patients, systemic sclerosis (SSc) patients, Sjogren's syndrome (SS) patients and other connective tissue disease patients.According to the patient's condition,the participants plan to be treated with different immunosuppressive agents with/wo pirfenidone .Participants can choose to continue the study up to 36 months.The efficacy and safety of the treatment in CTD-ILD patients will be evaluated with lung function, quality of life / cardiopulmonary function assessment and other disease activity indices.

Inclusion Criteria:

• Patients who meet the following inclusion criteria will be eligible to participate in the study:

  1. Aged 18-80 years;
  2. In accordance with the diagnostic criteria of connective tissue disease-related pulmonary interstitial disease (CTD-ILD);The diagnosis of CTD is in line with the international classification standard of rheumatism (including inflammatory myopathy, systemic sclerosis, rheumatoid arthritis, sjogren's syndrome, systemic lupus erythematosus, mixed connective tissue disease, undifferentiated connective tissue disease);
  3. Subjects are willing to participate and use medication and follow-up time according to the treatment plan, and sign the informed consent;

Exclusion Criteria:

• Patients who meet any of the following criteria will be excluded from the study:

  1. Subjects have non-diffuse connective tissue disease or other arthritis other than RA, such as ANCA-relateed vasculitis, psoriatic arthritis, etc;

  2. Patients who have ILD with clear etiology, such as HIV, GVHD ；

  3. Combined viscera function significantly abnormal patient:

     1. Liver: AST, ALT >1.3ULN; Bilirubin >1.5 ULN; or previous diagnosis of viral hepatitis;
     2. Kidney: Creatinine clearance <30 mL/min;
     3. Lung: Airway obstruction (pre-bronchodilator FEV1/FVC <0.7); pleural effusion accounts for more than 20% of pleural effusion ;severe pulmonary infection or other clinically significant pulmonary abnormalities;
     4. Cardiovascular: Myocardial infarction or unstable angina within six months;
     5. Gastrointestinal tract: With active peptic ulcer or bleeding;
     6. Blood system: Severe anemia, decreased white blood cells and platelets
     7. Nervous system: Patients with mental disorders; cerebral thrombosis events within the past 1 year;

  4. Combined with poor prognosis of diseases, such as cancer, genetic diseases and so on;

  5. Women during pregnancy or lactation or childbearing age cannot ensure effective contraception;

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

  7. Any other major medical events beyond control;