摘要
目的 研究中国儿童肉芽肿性关节炎的临床特点、实验室及辅助检查、治疗用药及远期预后,使本病能早期诊断和及时治疗.方法 回顾性分析北京儿童医院2006-2013年住院的中国儿童肉芽肿性关节炎患者26例,归纳总结其临床表现、实验室检查、治疗用药及转归和随访结果.结果 患儿生后早期发病,最早者为生后1个月内,最晚者生后3岁6个月起病;临床表现为关节炎、皮疹、虹膜睫状体炎,肝脾淋巴结肿大、大动脉炎、神经性耳聋和肾脏肉芽肿和1例中枢神经系统脱髓鞘病变;实验室检查可见轻度贫血,炎性指标不同程度升高,自身抗体检测均为阴性;所有患儿病理检查提示有多核巨细胞形成的非干酪样肉芽肿;NSAIDs联合糖皮质激素和甲氨蝶呤是基本用药,对于有虹膜睫状体炎和大动脉炎的患者早期使用TNF-α抑制剂效果较好;如规律治疗和随访,病情控制基本稳定,2例合并严重虹膜睫状体炎的患几经积极治疗视力有所恢复.结论 儿童肉芽肿性关节炎是一种早期发病的遗传性自身炎症性疾病,早期诊断和积极治疗可明显改善预后.
Objective To analyze the clinical features,laboratory data,treatment and outcome of patients with granulomatous arthritis of Chinese children.Methods Twenty-six children with granulomatous arthritis hospitalized in Beijing Children＇s Hospital between 2006 to 2013 were retrospectively analyzed.Results The onset age of granulomatous arthritis in these children was between 1 month and 3.5 years.The most common clinical features were arthritis,skin rash and iridocyclitis.Hepatomegaly,lymphadenopathy,Takayasu＇s arteritis,neurological deafness and renal granuloma were common clinical features.Demyelinating disease of central nervous system was also detected in one patient.Mild anemia,high-level inflammatory indexes,negative auto-antibody were found.The pathology of these children showed non-caseous granuloma with Warthin-Finkeldey giant cells.NSAIDs,glucocorticosteroid and methotrexate were basic drugs for treatment.Earlier use of TNF-α inhibitor for patients with iridocyclitis and arteritis revealed good effect.If regular treatment were initiated and followed-up regularly,patients could achieve satisfactory outcomes.The sight of two patients with iridocyclitis were improved after aggressive treatment.Conclusion Granulomatous arthritis of childhood is usually onset at early life and is a genetic auto-inflammatory disease,early diagnosis and active treatment can improve the prognosis.
出处
《中华风湿病学杂志》
CAS
CSCD
北大核心
2014年第9期593-596,I0002,共5页
Chinese Journal of Rheumatology
基金
中华医学会临床医学专项资金项目（12040690369）
关键词
症状和体征
随访研究
儿童肉芽肿性关节炎
Symptoms ＆ signs
Follow-up studies
Granulomatous arthritis of childhood
