Behcet's Syndrome
Conditions
Keywords
Behçet's syndrome、Ivarmacitinib
Brief summary
Behcet's syndrome (BS) is a multisystem autoimmune vasculitis. Current clinical treatment primarily includes glucocorticoids, immunosuppressants, and molecularly targeted drugs such as TNF-α and IL-6 inhibitors; however, some patients still respond poorly to existing treatment regimens. The JAK-STAT signaling pathway serves as a common downstream signaling pathway for various cytokines involved in the pathogenesis of Behcet's syndrome. Upon binding to their receptors, cytokines activate JAK kinases, which in turn phosphorylate STAT proteins to regulate the expression of inflammation-related genes. Therefore, blocking the JAK-STAT pathway can simultaneously inhibit the signal transduction of multiple pathogenic cytokines, thereby exerting anti-inflammatory effects. Consequently, Ivarmacitinib-a highly selective JAK1 inhibitor-holds promise for improving the prognosis and quality of life of patients with refractory Behçet's syndrome. This is a multi-center, single-arm trial conducted to evaluate the safety and efficacy of Ivarmacitinib in Behçet's syndrome (BS) . Patients with refractory Behçet's syndrome were enrolled . Patients received Ivarmacitinib for up to 24 weeks, which was added to the glucocorticoid and immunosuppressants. The clinical manifestations, inflammatory indicators, imaging and treatment of patients were recorded by investigators during the follow up.
Interventions
DRUGIvarmacitinib
The specific regimen was 4 mg of Ivarmacitinib administered daily for 24 weeks. All patients will undergo 24 weeks of prospective follow-up.
Sponsors
Liu Tian
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to 70 Years
Healthy volunteers
No
Inclusion criteria
* ① Meets the 1990 ISG diagnostic criteria for Behçet's syndrome \[9\]; * Aged 18-70 years; ③ Has shown an inadequate response to treatment after at least 6 months of therapy with glucocorticoids, at least two immunosuppressants, and/or biologics (including TNF-α inhibitors and IL-6 inhibitors); * Agrees to receive treatment with a new targeted therapy
Exclusion criteria
* ① Patients with one or more other autoimmune diseases; * Patients who have undergone major surgery within 4 weeks prior to enrollment, or who are scheduled to undergo elective surgery during the study; ③ Patients with a confirmed acute or chronic active infection (e.g., bacterial, viral \[such as EBV, CMV, HIV, or active hepatitis virus\]) within 4 weeks prior to enrollment; ④ Patients with a current or past history of any malignancy; * Female patients who are pregnant or within 6 months postpartum; * Patients with severe liver failure (Child-Pugh Class C) or end-stage renal disease (dialysis-dependent).
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Patients achieving complete remission and partial remission | Week 24 | The primary endpoint was defined as the proportion(percent) of patients in the whole cohort achieving complete remission and partial remission by week 24. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Changes of C-reactive protein | Week 24 | Blood samples were collected from all patients and the concentration of C-reactive protein (mg/L) were recorded. |
| Changes of erythrocyte sedimentation rate | week24 | Blood samples were collected from all patients and the erythrocyte sedimentation rates (mm/h) were recorded. |
| Changes of dosage of glucocorticoids from baseline. | week 24 | The dosage of glucocorticoids (mg/day) of all patients were recorded during the follow-up. |
Countries
China
Outcome results
None listed