Study of Treatment Response on IgG4 Related Disease (IgG4RD)

Study Overview
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Brief Summary
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This is an open-label randomized controlled trial to compare the efficacy of Prednisone alone and combination therapy of Prednisone and Mycophenolate mofetil in IgG4RD patients.

Detailed Description
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Patients with IgG4-RD will be randomized in two therapeutic groups: Prednisone alone and combination therapy with Prednisone and Mycophenolate mofetil. These patients will be followed in 12 months. Treatment response and relapse will be recorded, as well as side effects.

Official Title
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A Randomized Trial of Treatment in Patients With IgG4-Related Disease

Conditions
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Autoimmune Disease

Intervention / Treatment
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* Drug: Prednisone
* Drug: Prednisone and Mycophenolate mofetil


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Males and females Aged 18 to 70 years old with informed consent All patients must meet the following diagnostic criteria of IgG4RD (2011): swelling, sclerosing and inflammatory involvement of one or more organ, including sclerosing pancreatitis, sialadenitis (Mikulicz disease), sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, inflammatory aortic aneurysm, lymphadenopathy, or other inflammatory conditions; elevated serum IgG4 (>1.35 g/L); histopathologic features of fibrosis and/or lymphocytic and polyclonal plasma cell infiltration (and IgG4+ plasma cells on immunohistology when performed). Patients fulfill 1)+2)+3) are diagnosed as definite IgD4RD, 1)+2): possible IgG4RD; 1)+3): probable IgG4RD; exclusion of other diseases. Exclusion Criteria: Patients will not be included if meets any of the following criteria: Patients who were diagnosed as other autoimmune diseases; Patients who were diagnosed as malignant diseases; Pregnant and lactating women； Active infection: HIV, HCV, HBV, TB； Serious organ function failure, expected life time less than 6 months. Presenting with Mikulicz disease without other manifestations.

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
Allocation: Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Prednisone<br>Prednisone: started with prednisone 0. 6-0. 8mg/kg.d for 1 month, decreased 5mg per 2 weeks, maintained at 7.5mg to 10mg/d to 12 months. | Drug: Prednisone<br>* Prednisone: started with prednisone alone 0. 6-0. 8mg/kg.d for 1 month, decreased 5mg per 2 weeks, and maintained at 10mg/d to 12 months.<br>* Other names: Prednisolone;|
| Experimental: Prednisone and Mycophenolate mofetil<br>Prednisone: started with prednisone 0. 6-0. 8mg/kg.d for 1 month, decreased 5mg per 2 weeks, maintained at 7.5mg to 10mg/d to 12 months. Immunosuppressive drugs: Mycophenolate mofetil 1g/d-1.5g/d for 6 months and 0.5/d-1.0g/d for 6 months. | Drug: Prednisone and Mycophenolate mofetil<br>* Prednisone: started with prednisone 0. 6-0. 8mg/kg.d for 1 month, decreased 5mg per 2 weeks, maintained at 7.5mg to 10mg/d to 12 months. Immunosuppressive drugs: Mycophenolate mofetil 1g/d-1.5g/d for 6 months and 0.5/d-1.0g/d for 6 months.<br>* Other names: Mycophenolate mofetil Dispersible Tablets;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Complete and partial response rate at 3, 6, 9 and 12 months. | Complete and partial response are defined as resolution of clinical manifestations, biochemical tests (C-reactive Proteins and IgG or IgG4 levels), and imaging studies | Up to 12 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Disease response at 3, 6, 9 and 12 months. | Disease response is measured by IgG4-RD Responder Index(IgG4-RD RI) and defined as: Improvement of >2 points in the IgG4-RD RI over baseline No disease flares, as assessed by the IgG4-RD RI. | Up to 12 months | 
| Number of participants with adverse effect | Treatment-related adverse effect, including glucocorticoid-induced diabetes mellitus and infections. | Up to 12 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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IgG4 related disease, IgG4-RD, Response rate, Relapse