CAJ | 학술논문

目的评价Bohan/Peter标准(B/P标准)与欧洲神经肌肉疾病中心(ENMC)标准对特发性炎性肌病分型诊断皮肌炎和多发性肌炎的准确性.方法回顾性收集86例初诊为特发性炎性肌病患者的临床、实验室及骨骼肌病理资料,分别用B/P标准与ENMC标准进行分型诊断,比较两个标准诊断皮肌炎和多发性肌炎的异同性.数据分析采用SPSS 13.0软件系统进行非参数检验(Mann Whitney U检验)和一致性检验(Kappa分析)方法.结果 B/P标准诊断皮肌炎37例,多发性肌炎49例；ENMC标准诊断皮肌炎46例,多发性肌炎仅14例,其余为嗜酸细胞性肌炎1例、疑诊散发性包涵体肌炎9例,未能分型者5例,肢带型肌营养不良2B型11例.Kappa分析检验两个标准诊断皮肌炎一致性较好(κ=0.79),诊断多发性肌炎一致性差(κ=0.26).结论 B/P标准对多发性肌炎存在过度诊断、误诊风险.ENMC标准纳入免疫病理,增加了临床与病理诊断的排除标准,其分型诊断准确性优于B/P标准.
목적 평개Bohan/Peter표준(B/P표준)여구주신경기육질병중심(ENMC)표준대특발성염성기병분형진단피기염화다발성기염적준학성.방법 회고성수집86례초진위특발성염성기병환자적림상、실험실급골격기병리자료,분별용B/P표준여ENMC표준진행분형진단,비교량개표준진단피기염화다발성기염적이동성.수거분석채용SPSS 13.0연건계통진행비삼수검험(Mann Whitney U검험)화일치성검험(Kappa분석)방법.결과 B/P표준진단피기염37례,다발성기염49례；ENMC표준진단피기염46례,다발성기염부14례,기여위기산세포성기염1례、의진산발성포함체기염9례,미능분형자5례,지대형기영양불량2B형11례.Kappa분석검험량개표준진단피기염일치성교호(κ=0.79),진단다발성기염일치성차(κ=0.26).결론 B/P표준대다발성기염존재과도진단、오진풍험.ENMC표준납입면역병리,증가료림상여병리진단적배제표준,기분형진단준학성우우B/P표준.
Objective To estimate the consistency between the diagnostic criteria for dermatomyositis (DM) and polymyositis (PM) developed by Bohan and Peter criteria (B/P criteria) and ENMC criteria.Methods The clinical,laboratory and pathological data from 86 patients who were initially diagnosed with idiopathic inflammatory myopathy were collected retrospectively.These patients were diagnosed according to B/P criteria and ENMC criteria,and the similarities and differences between these two criteria were compared.The data were analyzed with Mann Whitney U test and Kappa test by SPSS 13.0 software.Results Thirtyseven DM and 49 PM were diagnosed using B/P criteria.Forty-six DM and 14 PM were diagnosed using ENMC criteria,and 1 was diagnosed as eosinophilic myositis,9 were diagnosed as sporadic inclusion body myositis (sIBM),11 cases were diagnosed as limb-girdle muscular dystrophy type 2B,and the diagnosis of 5 patients could not be clarified.Agreement for DM between these two sets of criteria was very good by Kappa test (κ=0.79),but the corresponding between the two tests for PM was poor (κ=0.26).Conclusion Our study has demonstrated that B/P criteria may cause over-diagnosis and misdiagnosing for PM.ENMC criteria involves immunohistochemical pathology,stratified clinical and pathological exclusion criteria.The diagnostic accuracy of ENMC criteria is much improved.