To Evaluate the Clinical Efficacy of Iguratimod in the Treatment of IgG4 Related Disease (IgG4-RD) With Mild Symptom

Study Overview
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Brief Summary
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30 untreated IgG4 related disease (IgG4-RD) patients with mild symptom are enrolled in this study, and will be treated with one dose of diprospan,then take Iguratimod 25mg, Bid orally. Patient's peripheral blood will be collected at baseline, 12 weeks and 24 weeks of follow up. The clinical efficacy will be evaluated by the IgG4-RD responder index, serum immunoglobulin, IgG4 and IgE, cytokines, and peripheral blood T cell and B cell sub-populations will be measured at baseline and follow up.

Detailed Description
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Glucocorticoid is the recognized first-line medication for IgG4-RD, but it has many side effects. In order to avoid long time glucocorticoids intake, patients with mild disease will be treated with Iguratimod combined with one dose of diprospan. 30 untreated IgG4-RD patients with mild symptom will be enrolled in this study, and be treated with one dose of diprospan,then take Iguratimod 25mg, Bid orally. 5 ml of patients' peripheral blood will be collected at baseline, 12 weeks and 24 weeks of follow up. At baseline and each follow-up, the clinical efficacy of treatment will be evaluated by IgG4-RD responder index, serum immunoglobulin, IgG4, immunoglobulin E (IgE), serum cytokines, such as interleukin 1, 6, and tumor necrosis factor, will be measured by ELISA, peripheral blood T cell sub-populations, B cell sub-populations and plasma cells will be measured by flow-cytometry.

Official Title
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To Evaluate the Clinical Efficacy of Iguratimod in the Treatment of IgG4 Related Disease (IgG4-RD) With Mild Symptom

Conditions
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IgG4-related Disease

Intervention / Treatment
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* Drug: T 614


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Mikulicz disease, with/without nasosinusitis or lymph node swelling. Without internal organs affected, With slow disease progression. Exclusion Criteria: Vital organs related:including autoimmune pancreatitis,retroperitoneal fibrosis, sclerosing cholangitis, lung related, kidney affected, IgG4 related castleman's disease and hypophysitis， Combined with other connective disease, With tumors, Pregnancy or to be pregnant, Active infection, including hepatitis B virus, hepatitis C virus, and tuberculosis. Leucocytopenia, impairment of liver and kidney function, Allergy of Iguratimod, or cannot tolerate Iguratimod.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 70 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Interventional Model Description: To compare IgG4-RD responder index, serum parameters and blood lymphocytes subpopulations at baseline and after treatment of 12 weeks and 24 weeks
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Signal arm study<br>Patients with mild symptom IgG4-RD are enrolled and inject one dosage of diprospan ,then take Iguratimod (T614), 25mg, Bid orally for three months. Firstly, we evaluate IgG4-RD responder index of patients at baseline and follow-up time.We collect the laboratory parameters and blood for lymphocytes subpopulations by flowcytometry. | Drug: T 614<br>* Patients of IgG4-RD with mild symptoms are given one dose of diprospan and Iguratimod, 25mg, Bid.<br>* Other names: diprospan;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| The change of IgG4-RD responder index before treatment and after treatment | Evaluate patients baseline and follow-up IgG4-RD responder index(disease activity) | 24 weeks | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Changes of serum immunoglobulins subclass IgG4 levels | The IgG4 levels are tested routinely. | 24 weeks | 
| Patients serum cytokines | patients serum of cytokines detection by ELISA. | 24 weeks | 
| Peripheral blood lymphocytes sub-populations | Peripheral blood T,B sub-populations by flowcytometry | 24 weeks | 
| Side effects | Side effects, including laboratory tests will be recorded and tested. | 24 weeks |