Ivarmacitinib for Refractory Behcet's Syndrome

Liu Tian

Overview

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.

Study Type

INTERVENTIONAL

Allocation

Purpose

TREATMENT

Masking

NONE

Conditions

Behcet's Syndrome

Interventions

Eligibility

Sex: ALLMin age: 18 YearsMax age: 70 Years

Medical Language ↔ Plain English

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).

Outcomes

Primary Outcomes

Patients achieving complete remission and partial remission

The primary endpoint was defined as the proportion(percent) of patients in the whole cohort achieving complete remission and partial remission by week 24.

Time frame: Week 24

Secondary Outcomes

Changes of C-reactive protein

Blood samples were collected from all patients and the concentration of C-reactive protein (mg/L) were recorded.

Time frame: Week 24

Changes of erythrocyte sedimentation rate

Blood samples were collected from all patients and the erythrocyte sedimentation rates (mm/h) were recorded.

Time frame: week24

Changes of dosage of glucocorticoids from baseline.

The dosage of glucocorticoids (mg/day) of all patients were recorded during the follow-up.

Time frame: week 24