Immunoglobulin G4–Related Disease: What an Allergist Should Know

Carballo, Iago; González‐Quintela, Arturo; Sopeña, B.; Vidal, Carmen

doi:10.18176/jiaci.0633

Cited by 6 publications

(5 citation statements)

References 131 publications

(326 reference statements)

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“…IgG4-RD is featured by often elevated levels of serum IgG4 and IgE ( 24 – 26 ). In line with previous studies, our findings also showed that the concentrations of serum IgG4 and IgE were significantly increased in IgG4-RD patients compared with HCs.…”

Section: Discussionmentioning

confidence: 99%

IL-7 Promotes the Expansion of Circulating CD28- Cytotoxic T Lymphocytes in Patients With IgG4-Related Disease via the JAK Signaling

Xia

Long

et al. 2022

Front. Immunol.

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ObjectivesThis study aimed to elucidate the changes and associated mechanisms of circulating CD28- cytotoxic T lymphocytes (CTLs) in patients with IgG4-related disease (IgG4-RD).MethodsFifty IgG4-RD patients and 15 healthy controls (HCs) were recruited. Peripheral blood mononuclear cells (PBMCs) were isolated, the levels of circulating CD28- CTLs were detected by flow cytometry, and the proportions of CD127lo or GZMB+CD28- CTL subsets were analyzed in the meantime. Mechanistically, PBMCs isolated from IgG4-RD patients were stimulated with IL-7 in the presence or absence of the JAK inhibitor tofacitinib. Flow cytometry was used to analyze the proliferation of CD28- CTLs and the changes in related subpopulations.ResultsCirculating CD4+CD28- CTLs and CD8+CD28- CTLs were significantly increased in IgG4-RD patients compared with HCs, accompanied by an elevation of CD127lo or GZMB+ CTL subsets. The ex vivo culture of PBMCs showed that IL-7 could induce the amplification of CD4+CD28- CTLs and CD8+CD28- CTLs in IgG4-RD. Furthermore, IL-7 promotes the proliferation and functional subset changes of these CD28- CTLs in this disease. The selective JAK inhibitor tofacitinib significantly inhibited the effects of IL-7 on CD4+CD28- CTLs and CD8+CD28- CTLs.ConclusionIL-7 can affect the immune balance of IgG4-RD patients by promoting the expansion and function of CD4+CD28- and CD8+CD28- CTLs in IgG4-RD through the JAK pathway. Blockade of the IL-7 signaling pathway may be a new therapeutic strategy for IgG4-RD.

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Section: Discussionmentioning

confidence: 99%

IL-7 Promotes the Expansion of Circulating CD28- Cytotoxic T Lymphocytes in Patients With IgG4-Related Disease via the JAK Signaling

Xia

Long

et al. 2022

Front. Immunol.

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“…Although the first cases were reported in Japan and other Asian countries, IgG-4-RD can affect all racial and ethnic groups even with a low reported prevalence. The mean onset age is 59 years, but some cases were described also in the paediatric population (Carballo et al, 2021). Most of the studies reported a slight male prevalence (around 56%), not always confirmed in most recent studies.…”

Section: Introductionmentioning

confidence: 90%

Update on Epidemiology of IgG4-related Disease Involving the Liver and Pancreas

Floreani

Gabbia²,

Martin³

2023

MRAJ

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IgG4-related disease (IgG4-RD) is a rare condition characterized by an immune-mediated fibro-inflammation affecting various organs (liver, pancreas, heart, kidney, brain among others) with peculiar histopathologic feature. Few epidemiological data have been published so far, although a dramatic increase in the number of patients diagnosed with IgG4-RD has been recorded in the last years. The clinical manifestations of IgG4-RD involve frequently liver and pancreas. Specifically, a crucial challenge in differential diagnosis is IgG4-related sclerosing cholangitis which is frequently accompanied by pancreatic involvement. Inflammatory alterations of liver parenchyma have also been described, with a new nosology of IgG4-autoimmune hepatitis. Type 1 autoimmune pancreatitis is the pancreatic manifestation of the IgG4-RD. The first-ever epidemiological study to estimate the point prevalence of IgG4-related sclerosing cholangitis has been recently conducted in Japan. Moreover, several demographic studies on IgG4-RD involving liver and pancreas have been published in other countries, although the majority of them are cohort studies and data on incidence/prevalence are lacking. This review aims to update the recent epidemiological and clinical knowledge of IgG4-RD involving liver and pancreas, focusing also on the risk of malignancy.

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“…Another differential diagnosis of iMCD, especially of the plasma cell type, is IgG4-RD, which is a systemic inflammatory disorder characterized by sclerosing inflammation rich in IgG4-expressing plasma cells. This disease frequently affects the pancreas, salivary glands and lymph nodes, but it can involve almost any tissue [ 64–70 ]. Misdiagnosis may be due to the fact that iMCD patients may have an elevated serum IgG4 level, while some cases of IgG4-RD may show Castleman-like histopathology.…”

Section: Inflammatory Conditions That Resemble Clinical or Histopatho...mentioning

confidence: 99%

Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

2022

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Idiopathic multicentric Castleman disease (iMCD) is an infrequent and life-threatening disorder characterised by systemic inflammatory symptoms, generalised lymphadenopathy, polyclonal lymphocyte proliferation, and organ dysfunction caused by a hyperinflammatory state. It accounts for one third to one half of all multicentric Castleman disease (MCD) cases. iMCD is often associated with autoimmune manifestations that may precede the iMCD diagnosis, be identified at the same time or follow it. In addition, iMCD may also coincide with a number of autoimmune diseases (such as psoriasis or myasthenia gravis) or autoinflammatory diseases (such as familial Mediterranean fever). Moreover, diverse inflammatory disorders, such as rheumatoid arthritis, systemic lupus erythematosus, adult-onset Still disease, systemic juvenile idiopathic arthritis, immunoglobulin (IgG4) related disease, or the recently described VEXAS syndrome, can present clinical features or lymphadenopathy with histopathological “Castleman-like” findings compatible with those of iMCD. Given the iMCD clinical heterogeneity and the overlap with other autoimmune or autoinflammatory disorders, iMCD diagnosis can be challenging. In this review, we explore the overlap between iMCD and inflammatory diseases and provide practical guidance on iMCD diagnosis in order to avoid misdiagnosis and confusion with other autoimmune or autoinflammatory conditions.

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Immunoglobulin G4–Related Disease: What an Allergist Should Know

Abstract: This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as

Cited by 6 publications

References 131 publications

IL-7 Promotes the Expansion of Circulating CD28- Cytotoxic T Lymphocytes in Patients With IgG4-Related Disease via the JAK Signaling

IL-7 Promotes the Expansion of Circulating CD28- Cytotoxic T Lymphocytes in Patients With IgG4-Related Disease via the JAK Signaling

Update on Epidemiology of IgG4-related Disease Involving the Liver and Pancreas

Idiopathic multicentric Castleman disease and associated autoimmune and autoinflammatory conditions: practical guidance for diagnosis

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