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Proliferative features of IgG4-related disease

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单位： [1]Rheumatology Unit Massachusetts General Hospital, Boston, MA, USA [2]Pancreatology Unit Massachusetts General Hospital, Boston, MA, USA [3]Center for Immunology and Inflammatory Diseases Massachusetts General Hospital, Boston, MA, USA [4]Harvard Medical School, Boston, MA, USA [5]San Raffaele Scientific Institute, Radiology, Milan, Italy [6]Royal Prince Alfred Hospital, Camperdown, NSW, Australia [7]School of Medical Sciences, The University of Sydney, Camperdown, NSW, Australia [8]Ragon Institute of Massachusetts Gneral Hospital, Massachusetts Institute of Technology, and Harvard University, Cambridge, MA, USA [9]Department of Rheumatology and Immunology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China

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摘要：

IgG4-related disease is an immune-mediated disease that can lead to substantial morbidity and organ damage. Capable of affecting nearly any organ system or anatomic site, and showing considerable overlap in clinical presentation with various other diseases, IgG4-related disease often poses a diagnostic challenge for clinicians. Furthermore, there are no diagnostic biomarkers with high specificity for IgG4-related disease, and histopathological examination is nuanced and requires clinical correlation for accurate diagnosis. Therefore, it is crucial for clinicians to recognise the clinical phenotypes of IgG4-related disease. The disease is generally considered to have predominantly fibrotic and proliferative (or inflammatory) manifestations, with distinct clinical, serological and histopathological findings associated with each manifestation. However, the fibrotic and proliferative manifestations of this disease frequently occur together, thereby blurring this dichotomous distinction. In this Series paper, we provide a detailed overview of the clinical manifestations typical of the proliferative features of IgG4-related disease, with an emphasis on the diagnostic evaluation and differential diagnosis of each proliferative disease manifestation. In addition, we summarise the immune mechanisms underlying IgG4-related disease, suggest a framework for how to approach management and monitoring after the diagnosis is established, and highlight current unmet needs for patient care surrounding this disease.Copyright © 2024 Elsevier Ltd. All rights reserved.

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出版当年[2023]版：

大类 | 1 区医学

小类 | 1 区风湿病学

最新[2025]版：

大类 | 1 区

医学

小类 | 1 区风湿病学

第一作者：

第一作者单位： [1]Rheumatology Unit Massachusetts General Hospital, Boston, MA, USA [4]Harvard Medical School, Boston, MA, USA

通讯作者：

通讯机构： [1]Rheumatology Unit Massachusetts General Hospital, Boston, MA, USA [3]Center for Immunology and Inflammatory Diseases Massachusetts General Hospital, Boston, MA, USA [4]Harvard Medical School, Boston, MA, USA [*1]Rheumatology Unit, Massachusetts General Hospital, Boston, MA 02114, USA

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