Abstract Background Behçet’s syndrome is a chronic, systemic vasculitis that affects multiple organs, with intestinal involvement referred to as intestinal Behçet’s disease. The understanding of effective treatments for this condition remains limited. Anti-tumor necrosis factor-α (TNF-α) therapy is recommended for moderate to severe or refractory cases. However, managing patients unresponsive to anti-TNF-α therapy presents a significant clinical challenge. Upadacitinib, a Janus kinase (JAK) inhibitor, has not previously been studied in this context. This study aims to evaluate the efficacy of upadacitinib in patients with anti-TNF-refractory intestinal Behçet’s disease. Methods We retrospectively included patients with refractory intestinal Behçet’s disease treated with upadacitinib at the First Affiliated Hospital of Zhejiang University School of Medicine. Data collected included clinical manifestations, medication history, physical examination findings, laboratory results, and endoscopic evaluations before and after treatment. Results Six patients with intestinal Behçet’s disease were included, five of whom were female, with an average age of 35.5±11.0 years. All patients presented with recurrent oral ulcers and abdominal symptoms, accompanied by endoscopic evidence of ileocecal ulcers. They had previously failed treatment with corticosteroids, immunosuppressants, and at least one anti-TNF-α biologic agent. Following 6.1±2.1 months of upadacitinib treatment, all patients showed clinical symptom improvement or relief. Compared to baseline, hemoglobin levels increased (107.2±11.3 vs. 127.5±7.9, P=0.030), serum albumin levels improved (39.2±4.0 vs. 47.2±1.4, P=0.009), C-reactive protein levels decreased (39.9±37.4 vs. 3.0±3.4, P=0.048), and Endoscopic performance improved (P=0.026). No serious adverse events were reported in either of the 6 patients during follow-up. Conclusion Upadacitinib appears to be a promising therapeutic option for patients with intestinal Behçet’s disease refractory to anti-TNF-α therapy.
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