中华风湿病学杂志
中華風濕病學雜誌
중화풍습병학잡지
CHINESE JOURNAL OF RHEUMATOLOGY
2014年
9期
602-606,652
,共6页
邓江红%李彩凤%韩彤昕%王江%邝伟英%周怡芳%张俊梅
鄧江紅%李綵鳳%韓彤昕%王江%鄺偉英%週怡芳%張俊梅
산강홍%리채봉%한동흔%왕강%광위영%주이방%장준매
儿童%硬皮病,局部性%硬皮病,系统性
兒童%硬皮病,跼部性%硬皮病,繫統性
인동%경피병,국부성%경피병,계통성
Child%Scleroderma,localized%Scleroderma,systemic
目的 分析儿童硬皮病临床及实验室检查特点.方法 对2002年1月到2013年10月在北京儿童医院风湿免疫科住院并诊断为儿童硬皮病的病例进行回顾性研究,按照国际上最新的硬皮病诊断标准重新进行评估,对患儿的流行病学及临床资料进行回顾性分析.定量资料2组间比较采用t检验.结果 共纳入46例患者,局限性硬皮病(LS)患者39例(85%),SSc患者7例(15%).LS患者平均起病年龄(5±4)岁,男女比例为1.2:1,SSc患者平均年龄(9±4)岁,均为女性.39例LS患者中,带状硬皮病21例(54%),混合性硬皮病14例(36%),广泛性硬斑病3例(8%),全硬化性硬斑病1例(3%).26例患者(67%)出现皮肤外脏器受累表现,3例神经系统受累见于创伤样硬皮病患儿.7例SSc患者均有脏器受累.以肺脏及消化道受累最多见,并可见心脏、神经系统及肾脏、眼科受累病例,1例硬皮病肾危象病例.实验室检查:30例(77%)LS患者ANA阳性,6例(15%)患者RF阳性,其中5例均有关节受累表现.7例(100%) SSc患者ANA均为高滴度阳性,5例(71%)患者Scl-70阳性.主要的治疗药物是甲氨蝶呤及泼尼松.结论 儿童局限性硬皮病更为常见,SSc引起脏器损害更重,多中心更大样本的研究有助于理解本病的全貌.
目的 分析兒童硬皮病臨床及實驗室檢查特點.方法 對2002年1月到2013年10月在北京兒童醫院風濕免疫科住院併診斷為兒童硬皮病的病例進行迴顧性研究,按照國際上最新的硬皮病診斷標準重新進行評估,對患兒的流行病學及臨床資料進行迴顧性分析.定量資料2組間比較採用t檢驗.結果 共納入46例患者,跼限性硬皮病(LS)患者39例(85%),SSc患者7例(15%).LS患者平均起病年齡(5±4)歲,男女比例為1.2:1,SSc患者平均年齡(9±4)歲,均為女性.39例LS患者中,帶狀硬皮病21例(54%),混閤性硬皮病14例(36%),廣汎性硬斑病3例(8%),全硬化性硬斑病1例(3%).26例患者(67%)齣現皮膚外髒器受纍錶現,3例神經繫統受纍見于創傷樣硬皮病患兒.7例SSc患者均有髒器受纍.以肺髒及消化道受纍最多見,併可見心髒、神經繫統及腎髒、眼科受纍病例,1例硬皮病腎危象病例.實驗室檢查:30例(77%)LS患者ANA暘性,6例(15%)患者RF暘性,其中5例均有關節受纍錶現.7例(100%) SSc患者ANA均為高滴度暘性,5例(71%)患者Scl-70暘性.主要的治療藥物是甲氨蝶呤及潑尼鬆.結論 兒童跼限性硬皮病更為常見,SSc引起髒器損害更重,多中心更大樣本的研究有助于理解本病的全貌.
목적 분석인동경피병림상급실험실검사특점.방법 대2002년1월도2013년10월재북경인동의원풍습면역과주원병진단위인동경피병적병례진행회고성연구,안조국제상최신적경피병진단표준중신진행평고,대환인적류행병학급림상자료진행회고성분석.정량자료2조간비교채용t검험.결과 공납입46례환자,국한성경피병(LS)환자39례(85%),SSc환자7례(15%).LS환자평균기병년령(5±4)세,남녀비례위1.2:1,SSc환자평균년령(9±4)세,균위녀성.39례LS환자중,대상경피병21례(54%),혼합성경피병14례(36%),엄범성경반병3례(8%),전경화성경반병1례(3%).26례환자(67%)출현피부외장기수루표현,3례신경계통수루견우창상양경피병환인.7례SSc환자균유장기수루.이폐장급소화도수루최다견,병가견심장、신경계통급신장、안과수루병례,1례경피병신위상병례.실험실검사:30례(77%)LS환자ANA양성,6례(15%)환자RF양성,기중5례균유관절수루표현.7례(100%) SSc환자ANA균위고적도양성,5례(71%)환자Scl-70양성.주요적치료약물시갑안접령급발니송.결론 인동국한성경피병경위상견,SSc인기장기손해경중,다중심경대양본적연구유조우리해본병적전모.
Objective To describe and analyze the clinical and laboratory findings in a group of children diagnosed with scleroderma at Beijing Children's Hospital in the last 10 years.Methods The clinical charts of children with scleroderma in the Rheumatology Department at Beijing Children's Hospital,between January 2002 and October 2013 were reviewed.All of them fulfilled the classification criteria for juvenile sclerodema,both systemic scleroderma (SSc) and localized scleroderma (LS) types.T test was used for comparison between the two groups.Results Forty-six patients were enrolled and were diagnosed as scleroderma.Seven patients(15%) suffered from SSc and 39 patients(85%) were LS.Mean age-at-onset of LS was (5±4) years old.The male to female ratio was 1.2:1.Mean age-at-onset of SSc was (9±4) years old.All patients were female.The lesions found in LS were linear scleroderma (54%),mixed morphea (36%),generalized morphea (8%),and panclerotic morphea (3%).Twenty-six patients had internal organs involved.Three patients with nerve system involvement was found in en coup de sabre (ECDS).Systemic involvement included lung and gastrointestinal tract primarily.The heart,nerve system,kidney,eye involvement was also found.One girl had SSc combined with renal crisis.Antinuclear antibodies were positive in 77% of LS patients and 100% of SSc patients.Rheumatic factor was positive in 6 patients (15%),5 patients had joint involvement.Tests for anti-Scl-70 antibodies were positive in 5 (71%) patients with SSc.The most common drugs used were methotrexate and prednisone.Conclusion In this study,LS is common in children.SSc is more severe than LS.Multi-center and large sample study is needed to know the characteristics of juvenile scleroderma in China.