Ustekinumab for Intestinal Behçet's Syndrome With Myelodysplastic Syndrome

Overview

Behçet's syndrome is a systemic vasculitis. Gastrointestinal involvement in Behçet's syndrome complicated by myelodysplastic syndrome represents a rare and severe subtype for which no standardized treatment guidelines currently exist, posing significant challenges in clinical practice. Ustekinumab, a biologic agent targeting IL-12/IL-23, has demonstrated favorable efficacy in both gastrointestinal Behçet's syndrome and inflammatory bowel disease. This study aims to evaluate the efficacy and safety of ustekinumab in patients with intestinal Behçet's syndrome and coexisting myelodysplastic syndrome.

This is a multicenter, single-arm clinical trial designed to evaluate the efficacy and safety of ustekinumab in patients with intestinal Behçet's syndrome complicated by myelodysplastic syndrome (MDS). A total of 8 patients with intestinal Behçet's syndrome and concomitant MDS will be enrolled. Prior to enrollment, all patients will discontinue any previous biologic agents. Ustekinumab will be administered subcutaneously at a dose of 90 mg at weeks 0, 4, and 8, followed by a maintenance dose of 90 mg every 12 weeks (every 3 months). All patients will be followed for a total of 24 months. Clinical manifestations, inflammatory biomarkers, and endoscopic findings will be documented throughout the study period. Concomitant medications will be recorded, and adverse events will be systematically monitored to evaluate the efficacy and safety of the treatment.

Conditions