IgG4-related Disease With Eosinophilia
Conditions
Keywords
IgG4-related disease, eosinophilia
Brief summary
Evaluation of efficacy and safety of Baricitinib combined with glucocorticoid in patients of IgG4-related disease with eosinophilia.
Detailed description
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.
Interventions
DRUGPrednisolone
Oral prednisolone 0.6-0.8mg/kg daily for 4 weeks, then tapered and withdrawal in 4 months.
DRUGBaricitinib
Oral Baricitinib 2mg daily for 12 months.
Sponsors
Tongji Hospital
Beijing Friendship Hospital
Wen Zhang
Study design
Allocation
RANDOMIZED
Intervention model
PARALLEL
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 70 Years
Healthy volunteers
No
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| 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
| Measure | Time frame | Description |
|---|---|---|
| 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 blood Eosinophil cells | 52 weeks | Percentages (%) and counts (109) of peripheral eosinophil cells |
| 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 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 ESR | 52 weeks | Serum erythrocyte sedimentation rate(mm/h) |
| The percentages of adverse events | 52 weeks | Adverse effect of drugs |
| The changes of PGA | 52 weeks | Changes of patient global assessment from baseline, score (0-10, higher is worse) |
| 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) |
Countries
China
Contacts
Primary ContactLinyi Peng, MD
Outcome results
None listed