中华医学杂志
中華醫學雜誌
중화의학잡지
National Medical Journal of China
2014年
41期
3259-3261
,共3页
李彩凤%王博玉%张俊梅%檀晓华
李綵鳳%王博玉%張俊梅%檀曉華
리채봉%왕박옥%장준매%단효화
红斑狼疮,系统性%临床研究%治疗结果%随访研究
紅斑狼瘡,繫統性%臨床研究%治療結果%隨訪研究
홍반랑창,계통성%림상연구%치료결과%수방연구
Systemic lupus erythematosus%Clinical features%Treatment%Follow-up
目的 总结儿童系统性红斑狼疮(SLE)患者临床特点、实验室表现及治疗转归.方法 回顾性分析2001至2008年在北京儿童医院首次确诊为SLE患儿130例的资料,研究其常见的首发表现、各系统受累发生率、血尿常规、主要自身抗体检查及治疗转归特点.130例中男25例女105例,男:女为1∶4.2,年龄4～16岁,平均(11.5±2.2)岁.结果 SLE患儿最常见的首发表现是发热(66.00％)、皮疹(62.33％)以及乏力不适(47.33％).皮肤黏膜(80.00％)、血液系统(70.77％)、肾脏(66.15％)是儿童SLE前3位易累及的器官.贫血(64.62％)和尿蛋白阳性(66.15％)是最常见的异常检查.自身抗体检查中ANA阳性率为100％,抗双链DNA抗体阳性率88.37％.6例继发抗磷脂综合征的SLE患儿IgM型抗心磷脂抗体和IgM型抗β2-糖蛋白-Ⅰ抗体均为中高度阳性.糖皮质激素是SLE患儿的首选用药,根据病情不同需要个体化用药.治疗后1个月有效率(缓解+好转)为80.7％(105/130),治疗1年后有效率为95.1％(97/102).治疗过程中死亡6例,均合并多脏器功能衰竭.结论 儿童SLE非特异性表现为最常见的首发表现,以重要脏器损害起病者预后差,皮肤黏膜、血液、肾脏是最易累及的系统,ANA多为高滴度阳性,监测IgM型抗心磷脂抗体和IgM型抗β2-糖蛋白-Ⅰ抗体可能有助于早期发现抗磷脂综合征,早期诊断、合理治疗、规律随诊可以改善病情转归.
目的 總結兒童繫統性紅斑狼瘡(SLE)患者臨床特點、實驗室錶現及治療轉歸.方法 迴顧性分析2001至2008年在北京兒童醫院首次確診為SLE患兒130例的資料,研究其常見的首髮錶現、各繫統受纍髮生率、血尿常規、主要自身抗體檢查及治療轉歸特點.130例中男25例女105例,男:女為1∶4.2,年齡4～16歲,平均(11.5±2.2)歲.結果 SLE患兒最常見的首髮錶現是髮熱(66.00％)、皮疹(62.33％)以及乏力不適(47.33％).皮膚黏膜(80.00％)、血液繫統(70.77％)、腎髒(66.15％)是兒童SLE前3位易纍及的器官.貧血(64.62％)和尿蛋白暘性(66.15％)是最常見的異常檢查.自身抗體檢查中ANA暘性率為100％,抗雙鏈DNA抗體暘性率88.37％.6例繼髮抗燐脂綜閤徵的SLE患兒IgM型抗心燐脂抗體和IgM型抗β2-糖蛋白-Ⅰ抗體均為中高度暘性.糖皮質激素是SLE患兒的首選用藥,根據病情不同需要箇體化用藥.治療後1箇月有效率(緩解+好轉)為80.7％(105/130),治療1年後有效率為95.1％(97/102).治療過程中死亡6例,均閤併多髒器功能衰竭.結論 兒童SLE非特異性錶現為最常見的首髮錶現,以重要髒器損害起病者預後差,皮膚黏膜、血液、腎髒是最易纍及的繫統,ANA多為高滴度暘性,鑑測IgM型抗心燐脂抗體和IgM型抗β2-糖蛋白-Ⅰ抗體可能有助于早期髮現抗燐脂綜閤徵,早期診斷、閤理治療、規律隨診可以改善病情轉歸.
목적 총결인동계통성홍반랑창(SLE)환자림상특점、실험실표현급치료전귀.방법 회고성분석2001지2008년재북경인동의원수차학진위SLE환인130례적자료,연구기상견적수발표현、각계통수루발생솔、혈뇨상규、주요자신항체검사급치료전귀특점.130례중남25례녀105례,남:녀위1∶4.2,년령4～16세,평균(11.5±2.2)세.결과 SLE환인최상견적수발표현시발열(66.00％)、피진(62.33％)이급핍력불괄(47.33％).피부점막(80.00％)、혈액계통(70.77％)、신장(66.15％)시인동SLE전3위역루급적기관.빈혈(64.62％)화뇨단백양성(66.15％)시최상견적이상검사.자신항체검사중ANA양성솔위100％,항쌍련DNA항체양성솔88.37％.6례계발항린지종합정적SLE환인IgM형항심린지항체화IgM형항β2-당단백-Ⅰ항체균위중고도양성.당피질격소시SLE환인적수선용약,근거병정불동수요개체화용약.치료후1개월유효솔(완해+호전)위80.7％(105/130),치료1년후유효솔위95.1％(97/102).치료과정중사망6례,균합병다장기공능쇠갈.결론 인동SLE비특이성표현위최상견적수발표현,이중요장기손해기병자예후차,피부점막、혈액、신장시최역루급적계통,ANA다위고적도양성,감측IgM형항심린지항체화IgM형항β2-당단백-Ⅰ항체가능유조우조기발현항린지종합정,조기진단、합리치료、규률수진가이개선병정전귀.
Objective To summarize the characteristics of clinical features,laboratory tests,treatments and outcomes of childhood systemic lupus erythematosus (SLE).Methods A retrospective study was conducted for 130 cases of first-confirmed childhood SLE hospitalized from 2001 to 2008.Their clinical data of initial manifestations,system involvements,laboratory tests,treatments and outcomes were recorded and analyzed.Among them,there were 25 males and 105 females with a gender ratio of 1 ∶ 4.2.The youngest was 4 years old and the oldest 16 years old.And the mean age of onset was 11.5 ±2.2 years.Results The initial manifestations included fever (66％),rash (62.33％) and fatigue (47.33％).And the cutaneous-mucosal system (80％),hematological system (70.77％) and kidney (66.15％) were frequently involved.Anemia (64.62％) and urine protein positivity (66.15％)were the most common abnormalities.All patients had positive ANA.The positive rate of anti-dsDNA was 88.37％.Six patients had antiphospholipid syndrome (APS).The levels of IgM-aCL and IgM-anti-β2-GP-Ⅰ were moderately or higher positive.Glucocorticoid was the first-line medication.All patients took steroids.Different immunodepressants were also used depending on the severity of illness.The effective rates of one month and one year were 80.7％ and 95.1％ respectively.Six patients died.Conclusion The patients with serious organ involvements as the initial manifestations end to have a worse prognosis.Cutaneous-mucosal system,hematological system and kidney are the most frequently involved.Most patients have a high level of ANA titer.IgM-aCL and IgM-anti-β2-GP-Ⅰ are sensitive for diagnosing antiphospholipid syndrome.Early diagnosis,appropriate treatment and regular follow-ups may help to improve the clinical prognosis of SLE.
