上海单中心住院儿童风湿性疾病谱演变

Spectrum change of rheumatic diseases in hospitalized children:a 12-year single-center experience of Shanghai

目的:了解上海单中心风湿性疾病住院患儿疾病谱的变化趋势,提高对儿童风湿性疾病的认识。方法:回顾性分析2005—2016年复旦大学附属儿科医院5950例患者的临床资料,采用χ2检验进行发生率的比较和分析。结果:①住院例数位列前3位的分别是:川崎病2633例(44.3%),过敏性紫癜(HSP)2109例(35.4%),幼年特发性关节炎(JIA)574例(9.6%)。②除外HLP,其余病种住院人数均呈上升趋势。③近6年住院的风湿性疾病种类由原来的17种增长到目前37种。④SLE患者逐年增长(112/2348和197/3602,χ2=1.41,P=0.235),重症狼疮患者数亦较前增多(35/112和55/197,χ2=0.38,P=0.536)。⑤风湿性疾病合并巨噬细胞活化综合征(MAS)的发生率为7.2‰(43/5950),幼年型关节炎伴全身发作(sJIA)中有12.9%(26/201)出现过MAS,占风湿性疾病合并MAS总数的60.5%(26/43)。近6年风湿性疾病合并MAS(χ2=14.1,P<0.01),及sJIA合并MAS均明显增多(χ2=11.2,P<0.01)。⑥1.1%(64/5950)风湿性疾病相关肺部病变,幼年型皮肌炎(JDM)中24.4%(20/82)合并风湿性疾病相关肺部病变,占风湿性疾病相关肺部病变总人数的31.3%(20/64)。近6年风湿性疾病并发相关肺部病变及患者明显增多(χ2=5.66,P=0.017)。⑦儿童风湿性疾病病死率为3.7‰(22/5950),45.5%发生于SLE(10/22)。近6年SLE病死率有所下降(5/112和5/197,χ2=0.34,P=0.558)。结论:风湿性疾病住院患者病种及人数由多到少依次为川崎病、HSP、JIA、SLE及JDM。在每年总患者数相对稳定情况下,少见、疑难、危重病种逐年增多。虽近6年SLE仍是风湿性疾病主要死因,但病死率逐年下降。

Objective To gain insight into the constitution of juvenile rheumatic diseases,treatment outcome and trends of rheumatic inpatients in past 12 years,and to improve awareness of juvenile rheumatic diseases.Methods The clinical data of 5950 patients in rheumatology department of the affiliated pediatric hospital of Fudan University(from 2005 to 2016)were analyzed retrospectively,and the chi-square test was used to compare and analyze the incidence.Results Disease changes:①The top three rheumatic diseases were Kawasaki disease(KD)(44.3%),Henoch-schoniein purpura(HSP)(35.4%),juvenile idiopathic arthritis(JIA)(9.6%).②The number of all constitution of juvenile rheumatic diseases in hospital increased other than HSP.③The rheumatic diseases were increased from 17 to 37 kinds in the past 6 years.④The number of systemic lupus erythematosus(SLE)increased year by year(112/2348 vs 197/3602,χ2=1.41,P=0.235),as well as the severe SLE(35/112 vs 55/197,χ2=0.38,P=0.536).⑤The rate of rheumatic diseases complicated with macrophage activation(MAS)was 7.2‰(43/5950).12.9%(26/201)of systemic juvenile idiopathic arthritis(sJIA)were complicated with MAS,which was accounted for 60.5%(26/43)of total number of MAS in rheumatic diseases.In the last 6 years,there was a significant increase in the number of patients with MAS in patients with rheumatic diseases(χ2=14.1,P<0.01)and sJIA(χ2=11.2,P<0.01).⑥1.1%(64/5950)of rheumatic diseases patients had lung lesions,juvenile dermatomyositis(JDM)accounted for 24.4%(20/82).In the last 6 years,the number of patients with lung lesions associated with rheumatic diseases increased significantly(χ2=5.66,P=0.017).⑦The mortality rate of juvenile rheumatic diseases was only 3.7‰(22/5950),and 45.5%occurred in SLE(10/22).The mortality rate of SLE decreased in last 6 years(5/112 vs 5/197,χ2=0.34,P=0.558).Conclusion The constitution of juvenile rheumatic diseases in our center is decreasing for systemic vasculitis(KD,HSP),JIA,SLE,JDM in last 6 years.The annual total number of patients is relatively stable.But rare,difficult and critically illed cases increase year by year.Although SLE is still the primary cause of death in juvenile rheumatic diseases in recent 6 years,the mortality rate has decreased year by year.