Sjögren's Syndrome
Conditions
Interventions
Telitacicept treatment group:Telitacicept 160 mg administered subcutaneously once weekly for 12 doses
Sponsors
West China Hospital, Sichuan University
Eligibility
Sex/Gender
All
Age
18 Years to 64 Years
Inclusion criteria
Inclusion criteria: 1. Age 18~64 years old; 2. Confirmed diagnosis of primary Sjögren's syndrome (pSS), which meets the 2016 ACR/EULAR international classification criteria (see Appendix 1); 3. Main manifestations of hematological system involvement: One of the following: (1) Leukopenia: peripheral blood leukocyte count is (2.0-3.0)×10^9/L; (2) Thrombocytopenia: platelet count is 50×10^9/L >= PLT >= 20×10^9/L; (3) Anemia: hemoglobin 35g/L 4. Ineffective previous treatment: patients who have been given prednisone or prednisolone for at least 4 weeks >=1 mg/kg/d with at least one immunosuppressive/modulator (cyclophosphamide, cyclosporine, mycophenolate mofetil, azathioprine, FK506, methotrexate, leflunomide and hydroxychloroquine, etc.) have not responded sustainably; or relapse after gradual reduction or discontinuation of glucocorticoids; 5. Good compliance: The patient has good compliance, can comply with the study protocol, and signs the informed consent form; 6. Stable treatment regimen: Stable treatment regimen for at least 28 days prior to the first dose of study drug. Standard therapy regimens are defined as stable use of any of the following (alone or in combination): glucocorticoids, antimalarials, other immunosuppressants or immunomodulators such as azathioprine, mycophenolate mofetil, cyclophosphamide, methotrexate, leflunomide, tacrolimus, cyclosporine, etc.
Exclusion criteria
Exclusion criteria: 1. Coexisting other systemic immune diseases: Patients will be excluded if they have other systemic autoimmune diseases that may mimic the pathology of primary Sjögren's syndrome (pSS) and could confound the study results. These include, but are not limited to: Systemic Lupus Erythematosus (SLE); Rheumatoid Arthritis (RA); Sarcoidosis; Antiphospholipid Syndrome; IgG4-Related Disease. 2. Life-threatening bleeding in vital organs (including, but not limited to, central nervous system hemorrhage, gastrointestinal bleeding), or intracranial hemorrhage within 6 months prior to screening. 3. Thrombotic thrombocytopenic purpura, hemolytic uremic syndrome, or thrombocytopenia due to other causes (e.g., sepsis, drugs). 4. Severe cardiovascular diseases: Unstable or uncontrolled conditions related to or affecting cardiac function (e.g., unstable angina, congestive heart failure, uncontrolled hypertension or arrhythmia); arteriovenous thromboembolic events; current use of antiplatelet or anticoagulant therapy; clinically significant electrocardiogram (ECG) changes; QTc >450 ms (males) or QTc >470 ms (females). 5. Severe pulmonary diseases: Unstable or uncontrolled conditions related to or affecting respiratory function [e.g., diffuse alveolar hemorrhage, severe pulmonary arterial hypertension, severe interstitial lung disease (peripheral oxygen saturation 6 g/24 hours or endogenous creatinine clearance rate =14 days. 7. Central nervous system diseases within 8 weeks prior to randomization (including epilepsy, psychosis, organic brain syndrome, cerebrovascular accident, encephalitis, central nervous system vasculitis). 8. Active hepatitis or significant past or present liver disease/history.Hepatitis B: Patients positive for HBsAg are excluded. Patients negative for HBsAg but positive for HBcAb require HBV-DNA testing: if HBV-DNA is positive, the patient is excluded; if HBV-DNA is negative, the patient is eligible.Hepatitis C: Patients positive for Hepatitis C antibody are excluded. 9. Exclusionary abnormal laboratory values include, but are not limited to: (1) ALT or AST >=3 × ULN (Upper Limit of Normal); (2) White blood cell count <1.5 × 10^9/L. 10. Immunodeficiency, active infection (e.g., herpes zoster, COVID-19, HIV virus, active tuberculosis, etc.), or active or recurrent peptic ulcer disease. 11. Pregnant or lactating women, or men or women planning to conceive during the trial period. 12. History of hypersensitivity to human-derived biological products. 13. Participation in any other interventional clinical trial within 28 days prior to randomization, or within 5 half-lives of the investigational product from a previous trial (whichever is longer); 14. Administration of any live vaccine within 28 days prior to randomization; 15. Use of thrombopoietin receptor agonists (TPO-RAs) (e.g., eltrombopag, romiplostim) within 28 days prior to randomization; 16. Patients with psychiatric disorders, including depression or active suicidal ideation/behavior; 17. Any other condition that, in the investigator’s judgment, would preclude safe participation in the study or interfere
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| White Blood Cell Count ;Platelet Count;Hemoglobin;Immunoglobulin;EULAR Sj?gren's Syndrome Disease Activity Index; | — |
Secondary
| Measure | Time frame |
|---|---|
| Patient-reported quality of life (assessed via SF-36 or other appropriate questionnaires);Daily Dose of Glucocorticoids; | — |
Countries
China
Contacts
Public ContactXiongyan Luo
West China Hospital, Sichuan University
Outcome results
None listed