关键词: Behcet’s disease, Children, 白塞病, 儿童

Abstract: Objective:To improve the diagnosis and treatment of Behcet's disease(BD), the clinical features of BD cases have been analyzed. Methods:Children with BD diagnosed in Children's Hospital, Capital Institute of Pediatrics from Aug 1, 2006 to Aug 1, 2016 were included. All patients were diagnosed according to 1990 International Behcet's Disease Diagnostic Criteria or 2014 International Criteria for Behcet's Disease. Results:Totally 27 cases were included, with 13 males and 14 females, aged from 8 months to 14 years(6.8±4.0 years). The median time from onset to diagnosis was 6 months. All of 27 children had oral ulcer. Gastrointestinal symptoms were found in 15 cases (55.6%), conjunctivitis and keratitis in 3 cases(11.1%)，rash in 14 cases (51.9%). Twenty cases undergoing endoscope examination all had positive endoscopic findings, 7 of whom did not have any gastrointestinal symptoms. Twenty-five cases received corticosteroids therapy. Among them, 8 cases only received methylprednisolone (MP), 10 cases received MP and CTX/MTX, 3 cases received MP, CTX and thalidomide, 1 case received MP and thalidomide, and 3 cases received MP and biologics. The other 2 cases were treated with total glucosides of paeony capsules. Four cases were lost to follow-up, and the other 23 cases were followed up for 36.2±39.1 months (0.5 months to 9 years), of whom 13 cases were stable, 7 cases relapsed and 3 cases died. Conclusion:The onset symptoms of BD in children were very obscure, and many children start with oral ulcers. Compared with BD adults, BD children were suffered less from eyes and skin symptoms, while digestive tract involvement was more common. After treatment, most of the BD children could be relieved and the prognosis is generally good. As to refractory cases, especially those who did not respond to treatment, primary immunodeficiency should be suspected.