IgG4-related disease
Conditions
Interventions
Experimental group:the combination of glucocorticoids and telitacicept
Control group :glucocorticoids
Sponsors
Zhongshan hospital, Fudan University
Eligibility
Sex/Gender
All
Age
18 Years to 70 Years
Inclusion criteria
Inclusion criteria: 1.Obtain written informed consent from participants. 2.Participants will be diagnosed based on the 2012 Japanese criteria or the 2019 ACR/EULAR criteria. Refer to the research protocol for further details. 3. Females or males aged between 18 and 70 years old; 4. All participants agree to have no plans for pregnancy during the clinical trial, and the serum or urine pregnancy test results for females must be negative; 5. Evidence of active disease within the past 3 months, meeting one of the major criteria or one or more minor criteria: 1)Major criterion: Imaging examination confirms new or worsened solitary or multiple swelling or single organ mass; 2)Minor criteria: i) New symptoms/signs related to IgG4-RD or worsening of previous symptoms/signs in the last month; ii) Elevated or higher than the last follow-up IgG4 levels; iii) Organ damage or deterioration occurs.
Exclusion criteria
Exclusion criteria: 1.Patients only presented with impaired organ function without evidence of clinical disease activity. 2.Subjects who had undergone complete surgical resection of the affected site. 3. Subjects with organ failure who meet at least one of the following criteria:I. GFR = 60 mL/min;II. Liver function Child-Pugh score C; 4. In addition to IgG4-RD, subjects have other autoimmune diseases as their main diagnosis (e.g., ANCA-associated vasculitis, systemic lupus erythematosus, rheumatoid arthritis, etc.); 5. The investigator believes that severe, progressive, or uncontrollable medical conditions unrelated to IgG4-RD involving the kidney, liver, blood system, gastrointestinal tract, lung, heart, nervous system, or other comorbidities may pose unacceptable risks to subjects participating in this study; 6. The investigator judges that a comorbidity (such as asthma) is likely to require additional use of medium to high-dose glucocorticoids (prednisone = 10 mg/day or equivalent dose of prednisone equivalents) during the study period; 7. Subjects with a history of malignant tumors; 8. Subjects with any serious acute or chronic infections; 9. Subjects positive for hepatitis B surface antigen or HBV DNA; 10. Subjects positive for HCV RNA; 11. Subjects are at risk of tuberculosis, especially if they have:i) Evidence from clinical, radiological, or laboratory tests indicating active or latent TB;ii) A history of active TB, even if treated; 12. Laboratory abnormalities meeting one or more of the following criteria:i) Serum ALT or AST >= three times the upper limit of normal;ii) Blood leukocyte count <= 3 × 10^9/L;iii) Platelet count <= 100 × 10^9/L;iv) Hemoglobin <= 80 g/L;v) Other laboratory findings that may pose unacceptable risks to subjects participating in this study; 13. Subjects allergic to any of the investigational drugs; 14. Prohibited treatments and/or medications: 1) subjects who have used tetatercept within 6 months; If corticosteroids are being used, the dose of prednisone should be < = 10 mg/day; 2) Within 4 weeks, the subject has used leflunomide, azathioprine, methotrexate, mycophenolate mofetil, cyclosporine, tacrolimus, thalidomide, eratimod, or any other drug for the treatment of IgG4-RD that is not specifically permitted by the protocol; 3) Received biologics such as rituximab, tumor necrosis factor inhibitors, etc. within 6 months; 4) Subjects who have received plasma exchange or lymphocyte exchange or immunosorbent therapy within 1 year or who have planned to receive such treatments; 5) Patients who are willing to receive attenuated vaccine during the trial; 6) Subjects who have received or plan to receive organ transplantation.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The response rate at 24 weeks; | — |
Secondary
| Measure | Time frame |
|---|---|
| Adverse event rate;Severe adverse event rate;The response rate at 12 weeks;IgG4-RD RI score;Doctor's global assessment;Subject self-report;Imaging findings;B cell subsets;Inflammation biomarker; | — |
Countries
China
Contacts
Public ContactLindi Jiang
Zhongshan hospital, Fudan University
Outcome results
None listed