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Clinical Reviews in Allergy & Immunology

Published in:

01-04-2018

The Immunologic Paradoxes of IgG4-Related Disease

Authors: Xiao Xiao, Min Lian, Weici Zhang, M. Eric Gershwin, Xiong Ma

Published in: Clinical Reviews in Allergy & Immunology | Issue 2/2018

Abstract

IgG4-related disease (IgG4-RD), which usually occurs in middle-aged and elderly men, is a newly recognized fibroinflammatory condition characterized by swelling and sclerosis of involved organs, increased IgG4-positive plasma cell infiltration in lesions, and elevated IgG4 concentration in serum. Despite growing interest in the research, the pathophysiological mechanism remains elusive. Most IgG4-RD patients respond well to steroid therapy initially, but recurrent and refractory cases are common, especially in advanced fibrotic stage. Recent studies have documented the heterogeneity of the B cell lineages, which suggests their multiple functions in IgG4-RD beyond IgG4 production, such as cytokine secretion, antigen presentation, autoantibody production, and modulation of T and B cell interactions. Thus, a critical balance exists between pathogenic and regulatory B subsets to prevent immunopathology. A prompt response to B cell depletion therapy reported in recent cases strongly suggests the imbalance within B cell lineages in IgG4-RD. A more precise understanding of the pathogenesis of IgG4-RD will open up new perspectives for therapeutic strategy. With a particular emphasis on the novel B cell-targeted therapeutic strategies, this review highlights the immunologic features of IgG4-RD and the possible roles of B cell lineages in the pathogenesis of IgG4-RD.

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Title: The Immunologic Paradoxes of IgG4-Related Disease
Authors: Xiao Xiao
Min Lian
Weici Zhang
M. Eric Gershwin
Xiong Ma
Publication date: 01-04-2018
Publisher: Springer US
Published in: Clinical Reviews in Allergy & Immunology / Issue 2/2018
Print ISSN: 1080-0549
Electronic ISSN: 1559-0267
DOI: https://doi.org/10.1007/s12016-018-8679-y

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At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

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Other articles of this Issue 2/2018

MicroRNAs: New Therapeutic Targets for Familial Hypercholesterolemia?

Review: Diagnosing Common Variable Immunodeficiency Disorder in the Era of Genome Sequencing

Inflammatory Joint Disorders and Neutrophilic Dermatoses: a Comprehensive Review

Effectiveness, Tolerability, and Safety of Belimumab in Patients with Refractory SLE: a Review of Observational Clinical-Practice-Based Studies

Anti-interleukin 5 Therapy for Eosinophilic Asthma: a Meta-analysis of Randomized Clinical Trials

Myeloid Cells and Chronic Liver Disease: a Comprehensive Review

ACC 2024 Congress Coverage

Year in Review: Heart failure and cardiomyopathies

Semaglutide benefits people with type 2 diabetes and obesity-related heart failure

Incentivised to exercise

New intervention for STEMI-related cardiogenic shock

» View more highlights on the ACC 2024 congress hub page