Interstitial Lung Disease Associated with Anti-Synthetase Syndrome
Conditions
Interventions
Telitacicept treatment group:Standard treatment + telitacicept 80 mg subcutaneous injection once a week
Standard treatment: Hormone combined with mycophenolate mofetil at a stable dose for 1 week
Sponsors
Rheumatology and Immunology Department, The First Affiliated Hospital of China Medical University
Eligibility
Sex/Gender
All
Age
18 Years to 70 Years
Inclusion criteria
Inclusion criteria: 1. Age: 18 to 70 years old (inclusive), gender not limited. 2. Those with a clear diagnosis of antisynthase syndrome by Connor's criteria. 3. The diagnosis of interstitial lung disease (ILD) was confirmed by high-resolution CT (HRCT) of the lungs. For patients who were initially treated or had relapsed or progressed after treatment, the doses of hormones and mycophenolate mofetil were stable one week before enrollment. 5. The patient voluntarily participated in this trial with good compliance and was capable of understanding and signing the informed consent form before the study.
Exclusion criteria
Exclusion criteria: 1. Anyone who does not meet any of the inclusion criteria. 2. Combined with other rheumatic diseases (excluding Sjogren's syndrome) : such as systemic sclerosis, systemic lupus erythematosus, mixed connective tissue disease, and undifferentiated connective tissue disease; Those with systemic vasculitis, such as ANCA-associated vasculitis; 3. Other diseases that may cause muscle lesions and symptoms of muscle weakness, including: neurological diseases (such as muscular dystrophy, myasthenia gravis, amyotrophic lateral sclerosis, Guillain-Barre syndrome), tumors, drugs (such as statins, etc.), infections, hereditary myopathy, endocrine myopathy, electrolyte imbalances, rhabdomyolysis; 4. The clinical history, physical signs, serological examination, HRCT and bronchoalveolar lavage results suggest that in addition to inflammatory myopathy, there are other diseases that can cause ILD, such as other CTD, systemic vasculitis, infection, tumor, sarcoidosis or allergic pneumonia caused by environmental factors. 5. Within the past three months, the CD20 antagonist rituximab, BAFF antagonist and other biological agents have been used. 6. Patients with significant organ function abnormalities: a) Liver: Alanine aminotransferase and aspartate aminotransferase > three times the upper limit of the normal range (excluding elevated aminotransferase caused by muscle lesions); Total bilirubin > 1.5 times the upper limit of the normal range; Those with liver cirrhosis classified as Child Pugh grade C; b) Kidneys: Creatinine clearance rate < 30 mL/min; c) Lungs: Patients with airway obstruction diseases (FEV1/FVC < 0.7 before the use of bronchodilators); Those with other seriously clinically significant abnormalities in the lungs; d) Cardiovascular: (i) Severe hypertension occurs within 6 weeks and is not controlled after treatment (=160/100 mmHg), (ii) Myocardial infarction occurs within 6 months, (iii) unstable angina pectoris occurs within 6 months; (iv) Those with pulmonary arterial hypertension accompanied by significant manifestations of right heart failure; (v) Those with myocardial involvement; e) Gastrointestinal tract: Those with active peptic ulcers; f) Nervous system: Patients with mental disorders; g) Coagulation function: A history of thrombotic events (including stroke and transient ischemic attack) within 12 months. 7. Researchers determine that patients whose life expectancy is less than one year due to other diseases (non-inflammatory myopathy, such as malignant tumors); 8. Allergic to the test drug or its components; 9. Patients who plan to undergo major surgery during the treatment period; 10. Women who are pregnant, breastfeeding or planning to become pregnant during the trial period. During the period 28 days before administration or 3 months after administration, women of childbearing age are unwilling or unable to use highly effective contraceptive methods. According to the researchers' view, the patient has a history of alcohol abuse or drug abuse. 13. Patients who cannot understand or follow the research procedures. 14. Researchers determine that patients have poor compliance and are difficult to complete the trial. 15. Patients with severe lower limb muscle weakness or joint lesions that affect the stability and endurance of walking and prevent them from conducting a 6-minute walk test; 16. There is a history of dysphagia or any gastrointestinal diseases that affect drug absorption; 17. Patients who are unable to cooperate to com
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Change of FVC%;The proportion of patients with no progression in the lungs at 24 weeks of treatment; | — |
Secondary
| Measure | Time frame |
|---|---|
| Changes in Dlco% of pulmonary function from baseline;The proportion and time of patients with DLco% decline > 15% compared with the baseline;The proportion and duration of patients with a decrease in FVC% greater than 10% compared to the baseline.;The area under the FVC% curve.;The changes in HRCT scores within 24 weeks compared to the baseline.;The change in 6-minute walking distance (6MWD) compared to the baseline.;The changes in the scores of the Health Status Questionnaire (SF-36) at the 24th week compared to the baseline.;The changes in the St. George's Respiratory Questionnaire (SGRQ) score within 24 weeks compared to the baseline.;The change in mMRC dyspnea score at week 24 compared to the baseline.;The time to overall mortality.;The change in TLC% within 24 weeks compared to the baseline.; | — |
Countries
China
Contacts
Public ContactXudong Liu
The First Affiliated Hospital of China Medical University
Outcome results
None listed