Leflunomide Treatment for IgG4-RD

Study Overview
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Brief Summary
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This study is an open-label randomized controlled trial aiming to investigate whether the efficacy and side effect of Leflunpomide plus glucocorticoid.

Official Title
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Leflunomide Treatment for IgG4-RD

Conditions
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Autoimmune Disease

Intervention / Treatment
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* Drug: Prednisone
* Drug: Prednisone and Leflunomide


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: Group 1<br>Patients treated with single glucocorticoid | 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 7.5mg to 10mg/d to 12 months.<br>Drug: Prednisone and Leflunomide<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. Leflunomide: 20mg qd for 6 months and 10mg qd for 6 months.<br>|
| Experimental: Group 2<br>Patients treated with Leflunomide and glucocorticoid | 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 7.5mg to 10mg/d to 12 months.<br>Drug: Prednisone and Leflunomide<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. Leflunomide: 20mg qd for 6 months and 10mg qd for 6 months.<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Response of Leflunomide treatment for IgG4-RD | Complete response rate; Partial response rate; No response | 1 year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Relapse of Leflunomide treatment for IgG4-RD | Clinical relapse; Serological relapse | 1 year |