Baricitinib Plus Glucocorticoid for Eosinophilia in IgG4-RD

Recruitment & Eligibility

Inclusion Criteria

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.
Active IgG4-RD (Responder Index ≥ 2 points for each involved organ)
The counts of peripheral blood eosinophil cells ≥0.75×109/L

Exclusion Criteria

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

Study & Design

Arm && Interventions

Group	Intervention	Description
Prednisolone monotherapy	Prednisolone	Oral prednisolone 0.6-0.8mg/kg daily for 4 weeks, then tapered and withdrawal in 4 months.
Prednisolone plus Baricitinib	Prednisolone	Oral prednisolone 0.6-0.8mg/kg daily for 4 weeks, then tapered and withdrawal in 4 months. Oral Baricitinib 2mg daily for 12 months.
Prednisolone plus Baricitinib	Baricitinib	Oral prednisolone 0.6-0.8mg/kg daily for 4 weeks, then tapered and withdrawal in 4 months. Oral Baricitinib 2mg daily for 12 months.

Primary Outcome Measures

Name	Time	Method
The difference of recurrent rate of IgG4-RD between the two groups	52 weeks	Clinical recurrence definition: any item of IgG4-RD Responder Index ≥2 (without treatment), or ≥ 3 (with treatment) or new organ involvement; with or without elevated serum IgG4 levels.

Secondary Outcome Measures

Name	Time	Method
The changes of serum IgG levels	52 weeks	Level of serum IgG(g/L)
The changes of serum IgG4 levels	52 weeks	Level of serum IgG4(mg/dL)
The changes of IgG4-related disease Responder Index	52 weeks	According to international multispecialty validation study of IgG4-related disease Responder Index (Version: 13, December, 2015), Responder Index ≥ 0, and higher scores mean a worse outcome.
The changes of ESR	52 weeks	Serum erythrocyte sedimentation rate(mm/h)
The percentages of adverse events	52 weeks	Adverse effect of drugs
The results of High-throughput analysis	52 weeks	The changes of results from baseline in High-throughput analysis, including GWAS, single cell sequencing of B cell receptor.
The changes of serum hsCRP level	52 weeks	Level of serum high-sensitivity C-reactive protein level(mg/L)
The changes of PGA	52 weeks	Changes of patient global assessment from baseline, score (0-10, higher is worse)
The time of recurrence	0-52 weeks	Clinical recurrence definition: any item of IgG4-RD Responder Index ≥2 (without treatment), or ≥ 3 (with treatment) or new organ involvement; with or without elevated serum IgG4 levels.
The changes of blood Eosinophil cells	52 weeks	Percentages (%) and counts (109) of peripheral eosinophil cells

Recruitment & Eligibility