Wang An, Fan Jie, Chen Xiaofeng, Wang Shaohua
Department of Thoracic Surgery, Huashan Hospital, Fudan University, Shanghai 200000, China.
Department of Pathology, Huashan Hospital, Fudan University, Shanghai 200000, China.
J Thorac Dis. 2018 Mar;10(3):1825-1841. doi: 10.21037/jtd.2018.01.149.
The existence of two diagnostic systems, the Boston and Japan criteria, for immunoglobulin G4-related disease (IgG4-RD) confuse the medical practice. We aimed to develop a comprehensive assessment based on the weight of each diagnostic item in the existing criteria to improve the diagnostic efficiency of Boston criteria.
We assessed the patients enrolled by a systematic review of the literatures using the Boston criteria, Japan criteria and a tentative comprehensive assessment respectively, and evaluated the efficiency of each system and their consistency.
Our analysis showed that the distinction in pathological diagnostic items was similar for the Boston criteria (IgG4+/IgG+ ratio, P<0.01; the number of pathological features and IgG4+ count, P<0.001) and comprehensive assessment (IgG4+/IgG+ ratio and the number of pathological features, P<0.001; IgG4+ count, P<0.05). For the Japan criteria, a good distinction in the number of pathological features was demonstrated (P<0.05) but the difference in the IgG4+/IgG+ ratio and IgG4+ count was not significant. There was relatively poor consistency between the Boston and Japan criteria (Kappa =0.482, P<0.001), while there was good agreement (Kappa =0.811, P<0.001), but a significant difference (P=0.011, McNemar matching test), between the Boston criteria and comprehensive assessment.
The current two diagnostic systems have poor consistency. Comprehensive assessment has good agreement with the Boston criteria, but can identify those cases in Boston Category 3 who could still be diagnosed as IgG4-related lung disease. Considering the weight of diagnostic items, the scoring system is a tentative exploration that should be improved with further experience in diagnosing IgG4-related lung disease.
免疫球蛋白G4相关疾病（IgG4-RD）存在波士顿标准和日本标准这两种诊断系统，这给临床实践带来了困惑。我们旨在基于现有标准中各诊断项目的权重制定一种综合评估方法，以提高波士顿标准的诊断效率。
我们分别使用波士顿标准、日本标准和一种初步的综合评估方法，对通过系统文献回顾纳入的患者进行评估，并评价每种系统的效率及其一致性。
我们的分析表明，波士顿标准（IgG4+/IgG+比值，P<0.01；病理特征数量和IgG4+计数，P<0.001）与综合评估（IgG4+/IgG+比值和病理特征数量，P<0.001；IgG4+计数，P<0.05）在病理诊断项目上的区分相似。对于日本标准，病理特征数量有显著区分（P<0.05），但IgG4+/IgG+比值和IgG4+计数的差异不显著。波士顿标准与日本标准之间的一致性相对较差（Kappa =0.482，P<0.001），而波士顿标准与综合评估之间有良好的一致性（Kappa =0.811，P<0.001），但存在显著差异（P=0.011，McNemar配对检验）。
目前的两种诊断系统一致性较差。综合评估与波士顿标准有良好的一致性，但能够识别出波士顿分类3类中仍可诊断为IgG4相关肺病的病例。考虑到诊断项目的权重，该评分系统是一种初步探索，应随着IgG4相关肺病诊断经验的积累进一步改进。
