Abstract：
OBJECTIVE: Our study aimed to investigate the distinct clinical patterns of seronegative IgG4-related disease (IgG4-RD) patients.
METHODS: We retrospectively enrolled 698 treatment-naïve IgG4-RD patients in this study. Patients were divided into four different subgroups according to their baseline serum IgG4 levels. The distinct clinical patterns of seronegative IgG4-RD patients were revealed through the comparison of baseline clinical data and disease prognosis among the different subgroups. COX regression analyses were used to investigate the risk factors for disease relapse and to construct the nomogram model.
RESULTS: Seronegative IgG4-RD patients account for a minority of IgG4-RD patients (49/698, 7.02%). The proportions of seronegative IgG-RD patients in our study and several Asian cohorts were significantly lower than those of the European and American cohorts. Seronegative IgG4-RD patients got lower serum IgG levels (p < 0.0001), lower eosinophil count (p < 0.0001), lower serum IgE levels (p < 0.0001)), lower IgG4-RD responder index (RI) scores (p < 0.0001), and fewer affected organ numbers (p < 0.0001) compared with other subgroups, whereas they were more likely to manifest fibrotic type with some special organ involvement. Younger age at onset, GCs monotherapy, elevated C-reactive protein level, and elevated erythrocyte sedimentation rate level are the risk factors for the disease relapse of seronegative IgG4-RD patients. An effective nomogram model predicting disease relapse of seronegative IgG4-RD patients was constructed. Seronegative IgG4-RD patients with scores >84.65 at baseline were susceptible to suffering from disease relapse.
CONCLUSIONS: Distinct clinical features and multiple risk factors for disease relapse of seronegative IgG4-RD patients have been revealed in this study. A nomogram model was constructed to effectively predict disease relapse during the follow-up period.
METHODS: We retrospectively enrolled 698 treatment-naïve IgG4-RD patients in this study. Patients were divided into four different subgroups according to their baseline serum IgG4 levels. The distinct clinical patterns of seronegative IgG4-RD patients were revealed through the comparison of baseline clinical data and disease prognosis among the different subgroups. COX regression analyses were used to investigate the risk factors for disease relapse and to construct the nomogram model.
RESULTS: Seronegative IgG4-RD patients account for a minority of IgG4-RD patients (49/698, 7.02%). The proportions of seronegative IgG-RD patients in our study and several Asian cohorts were significantly lower than those of the European and American cohorts. Seronegative IgG4-RD patients got lower serum IgG levels (p < 0.0001), lower eosinophil count (p < 0.0001), lower serum IgE levels (p < 0.0001)), lower IgG4-RD responder index (RI) scores (p < 0.0001), and fewer affected organ numbers (p < 0.0001) compared with other subgroups, whereas they were more likely to manifest fibrotic type with some special organ involvement. Younger age at onset, GCs monotherapy, elevated C-reactive protein level, and elevated erythrocyte sedimentation rate level are the risk factors for the disease relapse of seronegative IgG4-RD patients. An effective nomogram model predicting disease relapse of seronegative IgG4-RD patients was constructed. Seronegative IgG4-RD patients with scores >84.65 at baseline were susceptible to suffering from disease relapse.
CONCLUSIONS: Distinct clinical features and multiple risk factors for disease relapse of seronegative IgG4-RD patients have been revealed in this study. A nomogram model was constructed to effectively predict disease relapse during the follow-up period.
摘要：
目的：我们的研究旨在调查血清阴性IgG4相关疾病（IgG4-RD）患者的不同临床模式。
方法：在本研究中，我们回顾性地纳入了698例未接受治疗的IgG4-RD患者。根据患者的基线血清IgG4水平将患者分为四个不同的亚组。通过比较不同亚组之间的基线临床数据和疾病预后，揭示了血清阴性IgG4-RD患者的不同临床模式。COX回归分析用于研究疾病复发的危险因素并构建列线图模型。
结果：血清阴性IgG4-RD患者占IgG4-RD患者的少数（49/698，7.02％）。在我们的研究和几个亚洲队列中血清阴性IgG-RD患者的比例显着低于欧洲和美国队列。血清阴性IgG4-RD患者的血清IgG水平较低（p<0.0001），嗜酸性粒细胞计数降低（p<0.0001），降低血清IgE水平（p<0.0001）），较低的IgG4-RD反应指数（RI）得分（p<0.0001），与其他亚组相比，受影响的器官数量较少（p<0.0001），而他们更有可能表现为纤维化类型，有一些特殊的器官受累。发病年龄较小，GC单一疗法，C反应蛋白水平升高，血沉水平升高是血清阴性IgG4-RD患者疾病复发的危险因素。建立了预测血清阴性IgG4-RD患者疾病复发的有效列线图模型。基线评分>84.65的血清阴性IgG4-RD患者易患疾病复发。
结论：本研究揭示了血清阴性IgG4-RD患者疾病复发的不同临床特征和多种危险因素。建立了一个列线图模型来有效预测随访期间的疾病复发。
方法：在本研究中，我们回顾性地纳入了698例未接受治疗的IgG4-RD患者。根据患者的基线血清IgG4水平将患者分为四个不同的亚组。通过比较不同亚组之间的基线临床数据和疾病预后，揭示了血清阴性IgG4-RD患者的不同临床模式。COX回归分析用于研究疾病复发的危险因素并构建列线图模型。
结果：血清阴性IgG4-RD患者占IgG4-RD患者的少数（49/698，7.02％）。在我们的研究和几个亚洲队列中血清阴性IgG-RD患者的比例显着低于欧洲和美国队列。血清阴性IgG4-RD患者的血清IgG水平较低（p<0.0001），嗜酸性粒细胞计数降低（p<0.0001），降低血清IgE水平（p<0.0001）），较低的IgG4-RD反应指数（RI）得分（p<0.0001），与其他亚组相比，受影响的器官数量较少（p<0.0001），而他们更有可能表现为纤维化类型，有一些特殊的器官受累。发病年龄较小，GC单一疗法，C反应蛋白水平升高，血沉水平升高是血清阴性IgG4-RD患者疾病复发的危险因素。建立了预测血清阴性IgG4-RD患者疾病复发的有效列线图模型。基线评分>84.65的血清阴性IgG4-RD患者易患疾病复发。
结论：本研究揭示了血清阴性IgG4-RD患者疾病复发的不同临床特征和多种危险因素。建立了一个列线图模型来有效预测随访期间的疾病复发。
