Revealing the distinct clinical patterns and relapse risk factors in seronegative IgG4-RD patients: A retrospective cohort study over a decade

IF 9 2区医学 Q1 MEDICINE, GENERAL & INTERNAL

Journal of Internal Medicine Pub Date : 2024-06-24 DOI:10.1111/joim.13814

Yu Peng, Mu Wang, Ruijie Sun, Yuxue Nie, Nianyi Zhang, Xin He, Boyuan Sun, Linyi Peng, Yunyun Fei, Jiaxin Zhou, Mengtao Li, Wen Zhang

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引用次数: 0

Abstract

Objectives

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.

Abstract Image

查看原文本刊更多论文

揭示血清阴性 IgG4-RD 患者的独特临床模式和复发风险因素：一项历时十年的回顾性队列研究。

研究目的我们的研究旨在探讨血清阴性 IgG4 相关疾病（IgG4-RD）患者的独特临床模式：我们回顾性地纳入了 698 例未经治疗的 IgG4-RD 患者。根据基线血清 IgG4 水平，患者被分为四个不同的亚组。通过比较不同亚组的基线临床数据和疾病预后，揭示了血清阴性 IgG4-RD 患者不同的临床模式。COX回归分析用于研究疾病复发的风险因素并构建提名图模型：血清阴性IgG4-RD患者在IgG4-RD患者中占少数（49/698，7.02%）。在我们的研究和几个亚洲队列中，血清阴性 IgG-RD 患者的比例明显低于欧美队列。血清阴性 IgG4-RD 患者的血清 IgG 水平较低（p 84.65），容易复发：结论：本研究揭示了血清阴性 IgG4-RD 患者疾病复发的独特临床特征和多种风险因素。结论：本研究揭示了血清反应呈阴性的 IgG4-RD 患者复发的独特临床特征和多种风险因素，并构建了一个提名图模型，可有效预测随访期间的疾病复发。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of Internal Medicine 医学-医学：内科

CiteScore

22.00

自引率

0.90%

发文量

176

审稿时长

4-8 weeks

期刊介绍： JIM – The Journal of Internal Medicine, in continuous publication since 1863, is an international, peer-reviewed scientific journal. It publishes original work in clinical science, spanning from bench to bedside, encompassing a wide range of internal medicine and its subspecialties. JIM showcases original articles, reviews, brief reports, and research letters in the field of internal medicine.