CAJ | 학술논문

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.
목적 연구중국인동육아종성관절염적림상특점、실험실급보조검사、치료용약급원기예후,사본병능조기진단화급시치료.방법 회고성분석북경인동의원2006-2013년주원적중국인동육아종성관절염환자26례,귀납총결기림상표현、실험실검사、치료용약급전귀화수방결과.결과 환인생후조기발병,최조자위생후1개월내,최만자생후3세6개월기병;림상표현위관절염、피진、홍막첩상체염,간비림파결종대、대동맥염、신경성이롱화신장육아종화1례중추신경계통탈수초병변;실험실검사가견경도빈혈,염성지표불동정도승고,자신항체검측균위음성;소유환인병리검사제시유다핵거세포형성적비간락양육아종;NSAIDs연합당피질격소화갑안접령시기본용약,대우유홍막첩상체염화대동맥염적환자조기사용TNF-α억제제효과교호;여규률치료화수방,병정공제기본은정,2례합병엄중홍막첩상체염적환궤경적겁치료시력유소회복.결론 인동육아종성관절염시일충조기발병적유전성자신염증성질병,조기진단화적겁치료가명현개선예후.
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.