Clinical analysis of children Beh?et's disease with gastrointestinal involvement / 中华风湿病学杂志

ABSTRACT

Objective To summarize the clinical characteristics of Beh?et's disease (BD) in children with gastrointestinal involvement. Methods We retrospectively analyze the children BD with gastrointestinal involvement who were diagnosed in our hospital in recent 10 years. Results Twenty-two children were identified. The average age of onset was(6.1±4.0) years. The time from disease onset to clinical diagnosis was (1.2±2.1) years on average. Fifteen children had abdominal pain, diarrhea and hematochezia. Seven cases had positive endoscopic findings without any gastrointestinal symptoms. Twenty cases received corticosteroids therapy, 13 cases of them were treated with Cyclophosphamide/Methotrexate (CTX/MTX), 3 refractory cases were treated with biologics. Patients were followed up for (28±32) month on average. Eight patients' condition was stable, 7 patients were refractory, 3 patients died, 4 patients were lost to follow-up. At the same term, 5 patients without gastrointestinal involvement who received corticosteroids and CTX/MTX therapy were stable. Conclusion It is difficult to diagnose children BD at early stage. Gastrointestinal involvement may not be found, while the gastrointestinal endoscopy is of great importance in the diagnosis of the disease. Gluco-corticoid combined with immunosuppressive agents are effective. As to refractory patients, biological agent might be used although the recurrence is common. Compared with BD without gastrointestinal involvement, children BD with gastrointestinal involvement have serious condition and poor prognosis.