Differences in clinical characteristics of IgG4-related disease across age groups: a prospective study of 737 patients

Abstract Objective The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. Methods We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvem...

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Published inRheumatology (Oxford, England) Vol. 60; no. 6; pp. 2635 - 2646
Main Authors Lu, Hui, Teng, Fei, Zhang, Panpan, Fei, Yunyun, Peng, Linyi, Zhou, Jiaxin, Wang, Mu, Liu, Xiaowei, Zhu, Liang, Wang, Liwen, Luo, Xuan, Liu, Zheng, Li, Jieqiong, Zhao, Yan, Zhang, Wen, Zeng, Xiaofeng
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Published Oxford University Press 18.06.2021
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Abstract Abstract Objective The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. Methods We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40–49, 50–59, 60–69 and ≥70 years. The clinical characteristics of paediatric patients were also described. Results Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz’s disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse. Conclusion We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.
AbstractList The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups.OBJECTIVEThe aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups.We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40-49, 50-59, 60-69 and ≥70 years. The clinical characteristics of paediatric patients were also described.METHODSWe conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40-49, 50-59, 60-69 and ≥70 years. The clinical characteristics of paediatric patients were also described.Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz's disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse.RESULTSSex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz's disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse.We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.CONCLUSIONWe revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.
Abstract Objective The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. Methods We conducted a prospective study of 737 patients who were newly diagnosed with IgG4-RD and compared detailed demographic features, organ involvements, laboratory tests, treatments and outcomes across age groups. The patients were divided into five groups according to their age at diagnosis: ≤39, 40–49, 50–59, 60–69 and ≥70 years. The clinical characteristics of paediatric patients were also described. Results Sex ratio, disease duration, allergy history and clinical symptoms were significantly different across age groups. Besides, the proportions of superficial organ involvement (lacrimal gland and sinus) decreased with age, while the proportions of internal organ involvement (pancreas, biliary tract, retroperitoneal tissue, lung and prostate) increased with age, which was more prominent in male patients. Mikulicz’s disease was the most common manifestation (70%) in paediatric IgG4-RD patients. Multiple Cox analysis identified that age ≤56 years at diagnosis was an independent risk factor of relapse. Conclusion We revealed the impact of age on clinical characteristics of IgG4-RD, which indicated that different management might be required among different age groups.
Author Peng, Linyi
Lu, Hui
Zhang, Panpan
Wang, Mu
Fei, Yunyun
Liu, Xiaowei
Zhou, Jiaxin
Luo, Xuan
Zhu, Liang
Wang, Liwen
Zeng, Xiaofeng
Teng, Fei
Li, Jieqiong
Zhao, Yan
Zhang, Wen
Liu, Zheng
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  givenname: Hui
  surname: Lu
  fullname: Lu, Hui
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 2
  givenname: Fei
  surname: Teng
  fullname: Teng, Fei
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 3
  givenname: Panpan
  orcidid: 0000-0003-4120-8943
  surname: Zhang
  fullname: Zhang, Panpan
  email: zhangwen91@sina.com
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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  givenname: Yunyun
  orcidid: 0000-0003-1728-2342
  surname: Fei
  fullname: Fei, Yunyun
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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  givenname: Linyi
  surname: Peng
  fullname: Peng, Linyi
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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  givenname: Jiaxin
  surname: Zhou
  fullname: Zhou, Jiaxin
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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  givenname: Mu
  surname: Wang
  fullname: Wang, Mu
  organization: Department of Stomatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
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  givenname: Xiaowei
  surname: Liu
  fullname: Liu, Xiaowei
  organization: Department of Ophthalmology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
– sequence: 9
  givenname: Liang
  surname: Zhu
  fullname: Zhu, Liang
  organization: Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China
– sequence: 10
  givenname: Liwen
  orcidid: 0000-0003-2918-2402
  surname: Wang
  fullname: Wang, Liwen
  organization: Department of General Surgery, RuiJin Hospital affiliated to Shanghai Jiao Tong University School of medicine, Shanghai, China
– sequence: 11
  givenname: Xuan
  surname: Luo
  fullname: Luo, Xuan
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 12
  givenname: Zheng
  surname: Liu
  fullname: Liu, Zheng
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 13
  givenname: Jieqiong
  surname: Li
  fullname: Li, Jieqiong
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 14
  givenname: Yan
  surname: Zhao
  fullname: Zhao, Yan
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
– sequence: 15
  givenname: Wen
  surname: Zhang
  fullname: Zhang, Wen
  email: zhangwen91@sina.com
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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  givenname: Xiaofeng
  surname: Zeng
  fullname: Zeng, Xiaofeng
  organization: Department of Rheumatology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education & National Clinical Research Center for Dermatologic and Immunologic Diseases (NCRC-DID), Beijing, China
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The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.
Copyright_xml – notice: The Author(s) 2020. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com 2020
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Keywords paediatrics
IgG4-related disease
prognosis
age
clinical characteristics
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Snippet Abstract Objective The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups. Methods We...
The aim of this study was to compare the clinical characteristics of IgG4-related disease (IgG4-RD) among different age groups.OBJECTIVEThe aim of this study...
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Title Differences in clinical characteristics of IgG4-related disease across age groups: a prospective study of 737 patients
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