Clinical and pathological predictors of relapse in IgG4-related disease

Abstract Objectives In IgG4-related disease, the relationship between pathological findings and relapse has not been well established. This study aimed to identify the clinical and pathological predictors of disease relapse in IgG4-RD. Methods Patients with newly diagnosed IgG4-RD (n = 71) were enro...

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Main Authors: Ji Zongfei, Chen Lingli, Sun Ying, Ma Lingying, Zhang Lijuan, Liu Dongmei, Dai Xiaomin, Hou Yingyong, Chen Huiyong, Ma Lili, Jiang Lindi
Format: Article
Language:English
Published: BMC 2022-05-01
Series:Arthritis Research & Therapy
Subjects:
Online Access:https://doi.org/10.1186/s13075-022-02792-z
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author Ji Zongfei
Chen Lingli
Sun Ying
Ma Lingying
Zhang Lijuan
Liu Dongmei
Dai Xiaomin
Hou Yingyong
Chen Huiyong
Ma Lili
Jiang Lindi
author_facet Ji Zongfei
Chen Lingli
Sun Ying
Ma Lingying
Zhang Lijuan
Liu Dongmei
Dai Xiaomin
Hou Yingyong
Chen Huiyong
Ma Lili
Jiang Lindi
author_sort Ji Zongfei
collection DOAJ
description Abstract Objectives In IgG4-related disease, the relationship between pathological findings and relapse has not been well established. This study aimed to identify the clinical and pathological predictors of disease relapse in IgG4-RD. Methods Patients with newly diagnosed IgG4-RD (n = 71) were enrolled between January 2011 and April 2020; all cases were pathologically confirmed. The clinical and pathological features were recorded in a database at baseline and each follow-up visit. Patients were followed up at least once a month via outpatient clinic examinations and telephone calls. Univariate and multivariate Cox regression analyses and receiver operating curve (ROC) analysis were used to identify the predictors of disease relapse and to assess their predictive value. Results Over a median follow-up of 26 (range, 6–123) months, 3/71 (4.2%) patients died. Of the remaining 68 patients, 47 (69.1%) patients had achieved clinical remission and 21 (30.9%) had suffered relapse at the last follow-up. The independent predictors of relapse were IgG4 ≥ 6.5 g/L (HR = 2.84, 95% CI: 1.11–7.23), IgG ≥ 20.8 g/L (HR = 4.11, 95% CI: 1.53–11.06), IgG4-RD responder index (RI) ≥ 9 (HR = 3.82, 95% CI: 1.28–11.37), and severe IgG4+ plasma cell infiltration (HR = 6.32, 95% CI: 1.79–22.41). A prognostic score developed using three of the identified predictors (IgG ≥ 20.8 g/L, IgG4-RD RI ≥ 9, and severe IgG4+ plasma cell infiltration) showed good value for predicting impending relapse (AUC, 0.806). Conclusions In patients with IgG4-RD, IgG4 ≥ 6.5 g/L, IgG ≥ 20.8 g/L, IgG4-RD responder index (RI) ≥ 9, and severe IgG4+ plasma cell infiltration are predictors of relapse.
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spelling doaj.art-70918b9f9cb0449b88414cce6761489b2022-12-22T03:25:09ZengBMCArthritis Research & Therapy1478-63622022-05-012411810.1186/s13075-022-02792-zClinical and pathological predictors of relapse in IgG4-related diseaseJi Zongfei0Chen Lingli1Sun Ying2Ma Lingying3Zhang Lijuan4Liu Dongmei5Dai Xiaomin6Hou Yingyong7Chen Huiyong8Ma Lili9Jiang Lindi10Department of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Pathology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Xiamen Branch, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Pathology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityDepartment of Rheumatology, Zhongshan Hospital, Fudan UniversityAbstract Objectives In IgG4-related disease, the relationship between pathological findings and relapse has not been well established. This study aimed to identify the clinical and pathological predictors of disease relapse in IgG4-RD. Methods Patients with newly diagnosed IgG4-RD (n = 71) were enrolled between January 2011 and April 2020; all cases were pathologically confirmed. The clinical and pathological features were recorded in a database at baseline and each follow-up visit. Patients were followed up at least once a month via outpatient clinic examinations and telephone calls. Univariate and multivariate Cox regression analyses and receiver operating curve (ROC) analysis were used to identify the predictors of disease relapse and to assess their predictive value. Results Over a median follow-up of 26 (range, 6–123) months, 3/71 (4.2%) patients died. Of the remaining 68 patients, 47 (69.1%) patients had achieved clinical remission and 21 (30.9%) had suffered relapse at the last follow-up. The independent predictors of relapse were IgG4 ≥ 6.5 g/L (HR = 2.84, 95% CI: 1.11–7.23), IgG ≥ 20.8 g/L (HR = 4.11, 95% CI: 1.53–11.06), IgG4-RD responder index (RI) ≥ 9 (HR = 3.82, 95% CI: 1.28–11.37), and severe IgG4+ plasma cell infiltration (HR = 6.32, 95% CI: 1.79–22.41). A prognostic score developed using three of the identified predictors (IgG ≥ 20.8 g/L, IgG4-RD RI ≥ 9, and severe IgG4+ plasma cell infiltration) showed good value for predicting impending relapse (AUC, 0.806). Conclusions In patients with IgG4-RD, IgG4 ≥ 6.5 g/L, IgG ≥ 20.8 g/L, IgG4-RD responder index (RI) ≥ 9, and severe IgG4+ plasma cell infiltration are predictors of relapse.https://doi.org/10.1186/s13075-022-02792-zIgG4-related diseaseRelapsePrognostic factor
spellingShingle Ji Zongfei
Chen Lingli
Sun Ying
Ma Lingying
Zhang Lijuan
Liu Dongmei
Dai Xiaomin
Hou Yingyong
Chen Huiyong
Ma Lili
Jiang Lindi
Clinical and pathological predictors of relapse in IgG4-related disease
Arthritis Research & Therapy
IgG4-related disease
Relapse
Prognostic factor
title Clinical and pathological predictors of relapse in IgG4-related disease
title_full Clinical and pathological predictors of relapse in IgG4-related disease
title_fullStr Clinical and pathological predictors of relapse in IgG4-related disease
title_full_unstemmed Clinical and pathological predictors of relapse in IgG4-related disease
title_short Clinical and pathological predictors of relapse in IgG4-related disease
title_sort clinical and pathological predictors of relapse in igg4 related disease
topic IgG4-related disease
Relapse
Prognostic factor
url https://doi.org/10.1186/s13075-022-02792-z
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