Baricitinib Plus Glucocorticoid for Eosinophilia in IgG4-RD

A Prospective, Randomized Controlled Study to Compare the Efficacy and Safety of Baricitinib Combined With Glucocorticoid and Glucocorticoid Monotherapy in Proliferative IgG4-RD Patients With Eosinophilia

Evaluation of efficacy and safety of Baricitinib combined with glucocorticoid in patients of IgG4-related disease with eosinophilia.

This is a multicenter, 52-week prospective, randomized controlled study to compare the efficacy and safety of Baricitinib combined with glucocorticoid and glucocorticoid monotherapy in proliferative IgG4-RD patients with eosinophilia.

Inclusion Criteria:

1. All patients must meet the following diagnostic criteria of IgG4RD (2020): 1) and radiological features: One or more organs show diffuse or localized swelling or a mass or nodule characteristic of IgG4-RD. In single organ involvement, lymph node swelling is omitted. 2) serological diagnosis: Serum IgG4 levels greater than 135 mg/dl. 3) diagnosis diagnosis: Positivity for two of the following three criteria: a. Dense lymphocyte and plasma cell infiltration with fibrosis. b. Ratio of IgG4-positive plasma cells /IgG-positive cells greater than 40% and the number of IgG4-positive plasma cells greater than 10 per high powered field. c. Typical tissue fibrosis, particularly storiform fibrosis, or obliterative phlebitis.

   Patients fulfill (1) + (2) + (3) are diagnosed as definite IgD4RD; (1) + (2): possible IgG4RD; (1) + (3): probable IgG4RD. exclusion of other diseases.

2. Active IgG4-RD (Responder Index ≥ 2 points for each involved organ)

3. The counts of peripheral blood eosinophil cells ≥0.75×109/L

Exclusion Criteria:

1. Patients who is not able to discontinue GC
2. Pregnancy or breastfeeding or planning to get pregnant within 2 years
3. Received glucocorticoids, immunosuppressants, biological agents or JAK inhibitors within 3 months
4. Allergic to Baricitinib
5. Concomitant other autoimmune diseases
6. Malignancy
7. Chronic HBV infection, latent tuberculosis, or active infection
8. Server liver or renal dysfunction, or heart failure.