Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients

Abstract Background The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction. Methods We prospectively enrol...

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Main Authors: Liwen Wang, Panpan Zhang, Mu Wang, Ruie Feng, Yamin Lai, Linyi Peng, Yunyun Fei, Xuan Zhang, Yan Zhao, Xiaofeng Zeng, Fengchun Zhang, Wen Zhang
Format: Article
Language:English
Published: BMC 2018-04-01
Series:Arthritis Research & Therapy
Subjects:
Online Access:http://link.springer.com/article/10.1186/s13075-018-1567-2
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author Liwen Wang
Panpan Zhang
Mu Wang
Ruie Feng
Yamin Lai
Linyi Peng
Yunyun Fei
Xuan Zhang
Yan Zhao
Xiaofeng Zeng
Fengchun Zhang
Wen Zhang
author_facet Liwen Wang
Panpan Zhang
Mu Wang
Ruie Feng
Yamin Lai
Linyi Peng
Yunyun Fei
Xuan Zhang
Yan Zhao
Xiaofeng Zeng
Fengchun Zhang
Wen Zhang
author_sort Liwen Wang
collection DOAJ
description Abstract Background The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction. Methods We prospectively enrolled 215 newly diagnosed patients with IgG4-RD, who were initially treated with glucocorticoid (GC) alone or in combination with immunosuppressive agents (IM), and had at least 6 months of follow up. The therapeutic goals of remission induction were defined as fulfilling each of the following after the 6-month remission induction stage: (1) ≥ 50% decline in the IgG4-RD responder index (RI); (2) GC tapered to maintenance dose; and (3) no relapse during GC tapering. The patients not achieving the therapeutic goals were considered to have failed of remission induction. Results There were 26 patients in our cohort who failed of remission induction, including 16 (20.8%) on GC monotherapy, and 10 (7.2%) on combination therapy comprising GC and IM. The lacrimal gland and lung were most common sites of remission induction failure. Among the patients who relapsed during remission induction stage, 52.9% had secondary relapse during follow-up. Eosinophilia, higher baseline RI, more than five organs involved and dacryoadenitis were risk factors for remission induction failure with GC monotherapy, and the incidence of remission induction failure was 71.4% in the patients with more than three risk factors. After 6-month treatment, the patients who failed of remission induction had significantly higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and IgG4. Conclusion In our cohort, 20.8% of patients failed of remission induction with GC monotherapy, while 7.2% of patients failed of remission induction with combination therapy comprising GC and IM.
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spelling doaj.art-5cbc7579586d4687bb38182ce54ec3822022-12-21T18:56:27ZengBMCArthritis Research & Therapy1478-63622018-04-0120111210.1186/s13075-018-1567-2Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patientsLiwen Wang0Panpan Zhang1Mu Wang2Ruie Feng3Yamin Lai4Linyi Peng5Yunyun Fei6Xuan Zhang7Yan Zhao8Xiaofeng Zeng9Fengchun Zhang10Wen Zhang11Department of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Stomatology, Peking Union Medical College HospitalDepartment of Pathology, Peking Union Medical College HospitalDepartment of Gastroenterology, Peking Union Medical College HospitalDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationDepartment of Rheumatology, Peking Union Medical College Hospital (West Campus), Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of EducationAbstract Background The aim of this study was to assess the outcomes of remission induction in patients with IgG4-related disease (IgG4-RD) in our cohort, and to investigate the characteristics, prognosis, and risk factors in the patients failed of remission induction. Methods We prospectively enrolled 215 newly diagnosed patients with IgG4-RD, who were initially treated with glucocorticoid (GC) alone or in combination with immunosuppressive agents (IM), and had at least 6 months of follow up. The therapeutic goals of remission induction were defined as fulfilling each of the following after the 6-month remission induction stage: (1) ≥ 50% decline in the IgG4-RD responder index (RI); (2) GC tapered to maintenance dose; and (3) no relapse during GC tapering. The patients not achieving the therapeutic goals were considered to have failed of remission induction. Results There were 26 patients in our cohort who failed of remission induction, including 16 (20.8%) on GC monotherapy, and 10 (7.2%) on combination therapy comprising GC and IM. The lacrimal gland and lung were most common sites of remission induction failure. Among the patients who relapsed during remission induction stage, 52.9% had secondary relapse during follow-up. Eosinophilia, higher baseline RI, more than five organs involved and dacryoadenitis were risk factors for remission induction failure with GC monotherapy, and the incidence of remission induction failure was 71.4% in the patients with more than three risk factors. After 6-month treatment, the patients who failed of remission induction had significantly higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and IgG4. Conclusion In our cohort, 20.8% of patients failed of remission induction with GC monotherapy, while 7.2% of patients failed of remission induction with combination therapy comprising GC and IM.http://link.springer.com/article/10.1186/s13075-018-1567-2IgG4-related diseaseRemissionRelapseGlucocorticoidsImmunosuppressive agents
spellingShingle Liwen Wang
Panpan Zhang
Mu Wang
Ruie Feng
Yamin Lai
Linyi Peng
Yunyun Fei
Xuan Zhang
Yan Zhao
Xiaofeng Zeng
Fengchun Zhang
Wen Zhang
Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
Arthritis Research & Therapy
IgG4-related disease
Remission
Relapse
Glucocorticoids
Immunosuppressive agents
title Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
title_full Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
title_fullStr Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
title_full_unstemmed Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
title_short Failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in IgG4-related disease: a prospective study of 215 patients
title_sort failure of remission induction by glucocorticoids alone or in combination with immunosuppressive agents in igg4 related disease a prospective study of 215 patients
topic IgG4-related disease
Remission
Relapse
Glucocorticoids
Immunosuppressive agents
url http://link.springer.com/article/10.1186/s13075-018-1567-2
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