Glucocorticoids in Patients With IgG4-RD

Study Overview
=================
Brief Summary
-----------------
This is a randomized, open-label, single-center clinical trial to compare the efficacy and safety profile for medium-dose versus high dose glucocorticoid in patients with IgG4-related Disease. Patients will be followed for three months to measure the primary outcome and secondary outcomes.

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

Conditions
-----------------
IgG4-related Disease

Intervention / Treatment
-----------------
* Drug: Prednisone


Participation Criteria
=================
Eligibility Criteria
-----------------
Inclusion Criteria: Males and females Age 18-70 years old with informed consent Patients with IgG4-RD: swelling, sclerosing and inflammatory involvement of one or more organ, including sclerosing pancreatitis, sclerosing cholangitis, inflammatory pseudotumors, retroperitoneal or mediastinal fibrosis, interstitial nephritis, hypophysitis, sclerosing dacryoadenitis, sialadenitis, 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); exclusion of other diseases. Exclusion Criteria: Previously or currently received glucocorticoid and(or) immunomodulator Pregnancy or lactating Concurrent severe and/or uncontrolled and/or unstable diseases Patient with malignancy

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

Sexes Eligible for Study
-----------------
All

Accepts Healthy Volunteers
-----------------
No

Study Plan
=================
How is the study designed?
-----------------

Design Details

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


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Medium Dose<br>Patients are treated with prednisone or equivlent at doseage of 0.5-0.6 mg/kg/d (max 40mg daily) for 3 weeks, then tapering gradually to 15mg/d in 3 months. | Drug: Prednisone<br> <br> |
| Experimental: High Dose<br>Patients are treated with prednisone or equivlent at doseage of 0.8-1.0 mg/kg/d (max 60mg daily) for 3 weeks, then tapering gradually to 15mg/d in 3 months. | Drug: Prednisone<br> <br> |

What is the study measuring?
-----------------
Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Complete Response | Complete Response(CR) is defined as resolution of clinical manifestations, biochemical tests (C-reactive Proteins and IgG or IgG4 levels), and imaging studies. | 3 months | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Disease Response | 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. | 3 months | 
| Adverse Effect | Treatment-related adverse effect, including glucocorticoid-induced diabetes mellitus and infections. | 3 months | 

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