Behçet's syndrome (BS) is a systemic autoimmune vasculitis that can affect multiple organs, including the skin, eyes, and vascular system. Refractory BS poses significant treatment challenges, necessitating novel therapeutic approaches. Upadacitinib, a selective JAK1 inhibitor within the JAK-STAT pathway, has shown promise in modulating immune responses. This study aims to evaluate the efficacy and safety of upadacitinib in patients with refractory BS.
This multicenter, single-arm study investigates the efficacy and safety of upadacitinib (15 mg once daily) in refractory Behçet's syndrome (BS) patients. Adult patients had active BS with inadequate response to glucocorticoids and at least two conventional immunosuppressants or biologics over six months. Prior biologics were discontinued, and upadacitinib was added to ongoing glucocorticoids and immunosuppressants for 48 weeks. Clinical symptoms (oral/genital ulcers, skin lesions, uveitis), inflammatory markers (CRP, ESR), and medication usage were monitored. Adverse events were recorded to assess safety.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
27
All the BS patients discontinued other biologic agents and received oral upadacitinib treatment at a dose of 15mg per day with background glucocorticoids and immunosuppressants for 48 weeks. All the patients will be followed up prospectively for 48 weeks.
Department of Rheumatology and Immunology, Peking University People's Hospital
Beijing, Beijing Municipality, China
Patients getting improved condition
The primary endpoint was defined as the proportion(percent) of patients in the whole cohort getting improved condition by week 24. Improved condition was defined as BS-related manifestations resolved and no newly onset imaging/endoscopic findings observed.
Time frame: Week 24
Changes of Behcet's Disease Current Activity Form (BDCAF) score of patients
The clinical manifestation of patients were recorded during the follow-up. The disease activity of patients was accessed by Behcet's Disease Current Activity Form (BDCAF) score and the BDCAF scores (Range: 0\~12, higher scores mean higher disease activity) at week 24, week 48 and the baseline scores were compared.
Time frame: Week 24 and week 48
Changes of C-reactive protein
Blood samples were collected from all patients and the concentration of C-reactive protein (mg/L) were recorded. C-reactive protein at 24 weeks, 48 weeks and the baseline were compared.
Time frame: Week 24 and week 48
Changes of erythrocyte sedimentation rate
Blood samples were collected from all patients and the erythrocyte sedimentation rates (mm/h) were recorded. Erythrocyte sedimentation rates at 24 weeks, 48 weeks and the baseline were compared.
Time frame: Week 24 and week 48
Changes of dosage of glucocorticoids from baseline
The dosage of glucocorticoids (mg/day) of all patients were recorded during the follow-up. The dosage of glucocorticoids at 24 weeks, 48 weeks and the baseline were compared.
Time frame: Week 24 and week 48
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