Immunological mechanism of IgG4-related disease

Liu, Changyan; Zhang, Panpan; Zhang, Wen

doi:10.1016/j.jtauto.2020.100047

Cited by 30 publications

(42 citation statements)

References 72 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…T follicular helper (Tfh) cells are a subset of CD4+T cells that are known to be involved in the differentiation and class switch of B cells during their development. Among Tfh cells subsets, Tfh2 cells could induce the differentiation of naïve B cells into plasmablasts, subsequently promoting the production of IgG4 in active ( 48 ). Glucocorticoid-induced remission in patients with IgG4-RD is associated with a decrease of circulating CD4+ CTLs, a recently identified potentially pathogenic population of T lymphocytes ( 49 ).…”

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

View full text Add to dashboard Cite

Aims: To investigate the causes, clinical characteristics, imaging features, and therapeutic implications of hypertrophic pachymeningitis (HP) in a southern Chinese population. Methods: We retrospectively analyzed 48 patients with HP with different causes from 1 January 2006 to 31 December 2018. Clinical manifestation, laboratory findings, and neuroimaging results were evaluated in all HP patients. Results: The mean age at onset was 50 ± 12 years. The most common diagnosis was idiopathic HP (67%), followed by antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (15%), tuberculous meningitis (8%), viral meningitis (6%), and bacterial meningitis (4%). Headache was the most common symptom. The most frequently changed laboratory finding was elevated erythrocyte sedimentation rate (ESR). Imaging was characterized by cerebral or spinal dura mater enhancement in MRI scan with contrast. Enhancements were mainly located in the posterior fossa for idiopathic HP; frontal, parietal, and occipital lobes for ANCA-related HP; and posterior fossa for tuberculous-associated HP. Diffuse enhancement was found in most cases, except for tuberculous-associated HP. Glucocorticoid or immunosuppressive treatment was applied in most cases. Conclusions: The etiology of HP varied among patients, with idiopathic HP being the most common. MRI showed enhancement of the dura mater, which differed according to different etiologies. Glucocorticoid or immunosuppressive agents were the primary drugs for treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Xiao

2020

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…However, while ECD and IgG4-RD share some physiopathological characteristics, these diseases exhibit distinctive clinical features, suggesting they are distinct disorders. The increased production of IgG4 is frequently driven through a Th2 response to IL-4, IL-5, or Il-13 and by anti-inflammatory IL-10 and TGFβ cytokines 22 . Although ECD patients exhibit a Th1 immune response 4,6 , the present study suggested that while patients who carry the BRAF mutation exhibit higher circulating IL-10 concentrations and are more likely to exhibit high IgG4 levels than patients who lack the mutation, , suggesting that IL-10 might contribute to the IgG4 immune response in ECD.…”

Section: Discussionmentioning

confidence: 99%

Profound systemic alteration of the immune phenotype and an immunoglobulin switch in Erdheim-Chester disease in 78 patients from a single center

et al. 2021

View full text Add to dashboard Cite

Erdheim–Chester disease (ECD) is a rare, systemic, non-Langerhans cell histiocytosis neoplasm, which is characterized by the infiltration of CD63+ CD1a- histiocytes in multiple tissues. The BRAFV600E mutation is frequently present in individuals with ECD and has been detected in hematopoietic stem cells and immune cells from the myeloid and systemic compartments. Immune cells and proinflammatory cytokines are present in lesions, suggesting ECD involves immune cell recruitment. Although a systemic cytokine Th-1-oriented signature has been reported in ECD, the immune cell network orchestrating the immune response in ECD has yet to be described. To address this question, the phenotypes of circulating leukocytes were investigated in a large, single-center cohort of 78 patients with ECD and compared with a group of 21 control individuals. Major perturbations in the abundance of systemic immune cells were detected in patients with ECD, with a decrease in circulating plasmacytoid, myeloid 1, and myeloid 2 dendritic cells, mostly in BRAFV600E carriers, in comparison with individuals in the control group. Similarly, a marked decrease in blood T-helper, cytotoxic, and B lymphocyte numbers was observed in patients with ECD, relative to the control group. Measurement of circulating immunoglobulin concentrations revealed an immunoglobulin G switch, from IgG1 to IgG4 subclasses, which are more frequently associated with the BRAF mutation. First-line therapies, including pegylated IFNlland vemurafenib, were able to correct most of these alterations. This study reports a profound disturbance in the systemic immune phenotype in patients with ECD, providing important new information and helping to understand the physiopathological mechanisms involved in this rare disease and in the therapeutic management of patients.

show abstract

“…BAFF promotes immunoglobulin class switching, whereas IL-13 maintains Th2-cell dominant immune responses ( 68 ) ( Figure 1 ). Thus, activated basophils may induce IgG4 production via TLR signaling ( 69 ) ( Table 1 ). The specific TLR ligands that activate basophils in IgG4-RD patients are unknown.…”

Section: Type 2 Immunity In Igg4-rdmentioning

confidence: 99%

Allergic Aspects of IgG4-Related Disease: Implications for Pathogenesis and Therapy

et al. 2021

View full text Add to dashboard Cite

IgG4-related disease (IgG4-RD) is a rare systemic fibroinflammatory disease frequently associated with allergy. The pathogenesis of IgG4-RD is poorly understood, and effective therapies are limited. However, IgG4-RD appears to involve some of the same pathogenic mechanisms observed in allergic disease, such as T helper 2 (Th2) and regulatory T cell (Treg) activation, IgG4 and IgE hypersecretion, and blood/tissue eosinophilia. In addition, IgG4-RD tissue fibrosis appears to involve activation of basophils and mast cells and their release of alarmins and cytokines. In this article, we review allergy-like features of IgG4-RD and highlight targeted therapies for allergy that have potential in treating patients with IgG4-RD.

show abstract

Immunological mechanism of IgG4-related disease

Cited by 30 publications

References 72 publications

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Hypertrophic Pachymeningitis in a Southern Chinese Population: A Retrospective Study

Profound systemic alteration of the immune phenotype and an immunoglobulin switch in Erdheim-Chester disease in 78 patients from a single center

Allergic Aspects of IgG4-Related Disease: Implications for Pathogenesis and Therapy

Contact Info

Product

Resources

About