Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts
Clinical phenotypes of IgG4-related disease: an analysis of two international cross-sectional cohorts
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DOI：
10.1136/annrheumdis-2018-214603
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发表时间：
2019-03-01
影响因子：
27.4
通讯作者：
Zhang, Wen
中科院分区：
文献类型：
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作者：
Wallace, Zachary S.;Zhang, Yuqing;Zhang, Wen
目的：igg4相关疾病（IgG4-RD）是一种病因不明的异质、多器官疾病，可导致器官衰竭。难以识别IgG4-RD导致诊断延迟。我们试图确定关键的IgG4-RD表型。方法：我们使用了两项横断面研究，这些研究由提交了765例IgG4-RD专家组成的国际多专业网络集合，以推导和复制表型组。疾病表现的表型组和鉴定组间的关键协变量分布使用潜在类分析进行测量。结果在衍生队列（n=493）中，我们确定了四组具有不同表现的患者：1组（31%），胰脏-肝脏-胆道疾病；2组（24%），腹膜后纤维化和/或主动脉炎；第3组（24%）为头颈部受限疾病，第4组（22%）为全身受累的经典Mikulicz综合征。我们在复制队列中重复鉴定了四个表型组。与第1、2和4组的病例相比，第3组的病例更可能是女性（OR为11.60 （95% CI 5.39 ~ 24.98）、10.35 （95% CI 4.63 ~ 23.15）和9.24 (95% CI 3.53 ~ 24.20)）和亚洲人（OR为6.68 （95% CI 2.82 ~ 15.79）、7.43 （95% CI 2.97 ~ 18.56）和6.27 (95% CI 2.27 ~ 17.29)）。第4组患者血清IgG4中位浓度（1170 mg/dL）高于第1-3组（分别为316、178和445 mg/dL）
Objective I gG4-related disease (IgG4-RD) is a heterogeneous, multiorgan condition of unclear aetiology that can cause organ failure. Difficulty recognising IgG4-RD contributes to diagnostic delays. We sought to identify key IgG4-RD phenotypes.Methods We used two cross-sectional studies assembled by an international, multispecialty network of IgG4-RD specialists who submitted 765 cases to derive and replicate phenotypic groups. Phenotype groups of disease manifestations and key covariate distributions across the identified groups were measured using latent class analysis.Results In the derivation cohort (n=493), we identified four groups with distinct manifestations: Group 1 (31%), Pancreato-Hepato-Biliary disease; Group 2 (24%), Retroperitoneal Fibrosis and/or Aortitis; Group 3 (24%), Head and Neck-Limited disease and Group 4 (22%), classic Mikulicz syndrome with systemic involvement. We replicated the identification of four phenotype groups in the replication cohort. Compared with cases in Groups 1, 2 and 4, respectively, cases in Group 3 were more likely to be female (OR 11.60 (95% CI 5.39 to 24.98), 10.35 (95% CI 4.63 to 23.15) and 9.24 (95% CI 3.53 to 24.20)) and Asian (OR 6.68 (95% CI 2.82 to 15.79), 7.43 (95% CI 2.97 to 18.56) and 6.27 (95% CI 2.27 to 17.29)). Cases in Group 4 had a higher median serum IgG4 concentration (1170 mg/dL) compared with groups 1-3 (316, 178 and 445 mg/dL, respectively, p