Immunoglobulin G4-Related Disease: An Update

Al‐Mujaini, Abdullah; Al-Khabori, Murtadha; Shenoy, Kashinatha; Wali, Upender

doi:10.5001/omj.2018.20

Cited by 21 publications

(32 citation statements)

References 58 publications

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“…Immunoglobulin G4-related disease (IgG4-RD) is a newly defined immune-mediated disease with common clinical, serological, and pathological features ( 1 ). Common features of IgG4-RD include serum IgG4 level elevation, multiple organ involvements, dense infiltration of IgG4+ plasma cells, and significant tissue infiltrates ( 2 ). This disease affects men more often than women and age at diagnosis ranges from 50 to 70 years ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

Multiple Processes May Involve in the IgG4-RD Pathogenesis: An Integrative Study via Proteomic and Transcriptomic Analysis

et al. 2020

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Immunoglobulin G4-related disease (IgG4-RD) is a newly defined disease entity, while the exact pathogenesis is still not clear. Identifying the characters of IgG4-RD in proteomic and transcriptomic aspects will be critical to investigate the potential pathogenic mechanisms of IgG4-RD. We performed proteomic analysis realized with iTRAQ technique for serum samples from eight treatment-naive IgG4-RD patients and eight healthy volunteers, and tissue samples from two IgG4-RD patients and two non-IgG4-RD patients. Transcriptomic data (GSE40568 and GSE66465) was obtained from the GEO Dataset for validation. The weighted correlation network analysis (WGCNA) was applied to detect the gene modules correlated with IgG4-RD. KEGG pathway analysis was used to investigate pathways enriched in IgG4-RD samples. As a result, a total of 980 differentially expressed proteins (DEPs) in tissue and 94 DEPs in serum were identified between IgG4-RD and control groups. Three hundred fifty-four and two hundred forty-seven genes that most correlated with IgG4-RD were detected by WGCNA analysis in tissue and PBMC, respectively. We also found that DEPs in IgG4-RD samples were enriched in several immune-related activities including bacterial/viral infections and platelet activation as well as some immune related signaling pathways. In conclusion, we identified multiple processes/factors and several signaling pathways that may involve in the IgG4-RD pathogenesis, and found out some potential therapeutic targets for IgG4-RD.

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Section: Introductionmentioning

confidence: 99%

Multiple Processes May Involve in the IgG4-RD Pathogenesis: An Integrative Study via Proteomic and Transcriptomic Analysis

et al. 2020

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“…Though classically GPA- and IgG4-related diseases have been pathologically distinct, they have been described to have atypical presentations, including pachymeningitis, suggesting there is a clinical overlap between the two conditions. GPA predominantly produces a leukocytoclastic vasculitis with granulomatous inflammation with the typical presentation of pulmonary nodules and/or renal involvement, whereas IgG4-related diseases have been largely associated with lymphoplasmacytic infiltrates and pseudotumors that often manifest with inflammatory disease [3, 5]. The case above describes both GPA and IgG4-related disease which may represent disease pathogenesis to be a spectrum instead of two distinct processes.…”

Section: Discussionmentioning

confidence: 99%

“…The most common presenting features of HP are headache and cranial nerve dysfunction [1]. IgG4-related disease is a group of immune-mediated inflammatory diseases, commonly with multiple organ involvement, characterized by lymphoplasmacytic infiltration of IgG4-rich plasma cells, formation of swellings or masses resembling tumors, and elevated IgG4 levels in serum [2, 3]. Cases usually present with a mass or nodule in the orbit, kidney, or lungs; or diffuse enlargement of the pancreas.…”

Section: Introductionmentioning

confidence: 99%

“…Cases usually present with a mass or nodule in the orbit, kidney, or lungs; or diffuse enlargement of the pancreas. The disease is commonly associated with meningitis and cranial nerve dysfunction and may produce focal neurologic deficits by compressing nerves or vascular structures [3]. Presentation of IgG4-related HP is not usually distinguishable from other etiologies of HP; however, clinical manifestations beyond the meninges may be important in diagnosis [2].…”

Section: Introductionmentioning

confidence: 99%

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A Case of Hypertrophic Pachymeningitis Associated with Immunoglobulin-G4 and c-ANCA

Wyrostek¹,

Chakrabarti²,

Baldwin³

et al. 2019

Case Rep Neurol

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Hypertrophic pachymeningitis (HP) is characterized by inflammation of the dura mater. It has been described in the setting of numerous systemic inflammatory diseases including immunoglobulin G4 (IgG4)-related disease as well as granulomatosis with polyangiitis (GPA). In this case report, we describe a 48-year-old man presenting with headache who was found to have HP and had systemic features of both GPA and IgG4-related disease as well as seropositivity for both cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) and IgG4. He was treated with prednisone and rituximab with improvement in his symptoms. Co-occurrence of IgG4 and ANCA against myeloperoxidase has been reported in other cases of HP. The overlap between IgG4 and ANCA has also been described in other systemic manifestations of the diseases. These reports suggest a clinical overlap between ANCA and IgG4-related disease, and the case presented herein suggests an overlap between GPA and IgG4-related disease.

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“…IgG4-assoziierte Erkrankungen sind eine Gruppe von immunologisch vermittelten Erkrankungen, die verschiedene Organe betreffen können. Betroffen sein können typischerweise die Orbita, die Speicheldrüsen, die Lunge, die Bauchspeicheldrüse, die Gallengänge und das retroperitoneale Gewebe [ 1 , 2 ]. Die Kopf-Hals-Region ist am zweithäufigsten nach dem Pankreas betroffen [ 3 ].…”

Section: Hintergrundunclassified

IgG4-assoziierte Orbitopathie als wichtige Differenzialdiagnose eines fortgeschrittenen Silent-Sinus-Syndroms

Jurkov

Klauschen

Bertelmann

et al. 2020

HNO

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Background Immunoglobulin (Ig)G4-related disease is classified as an immune-mediated disease. The etiology of this condition has not been explained to date. Manifestations of the disease are diverse, and simultaneous involvement of multiple organs is not unusual. Case report We report the case of a patient referred to us after multiple unsuccessful paranasal sinus operations who presented with enophthalmos and a resultant migratory keratitis with a suspected diagnosis of silent sinus syndrome. Preservation of the orbit was no longer feasible. After five years without a definitive diagnosis, we ascertained that this was a case of IgG4-related disease. Discussion IgG4-related disease represents an important element in the differential diagnosis of chronic advanced diseases of the orbit and paranasal sinuses. The diagnosis should be considered in the case of unclear disease presentations. Typical histological findings include a storiform pattern of fibrosis, vasculopathy, and tissue infiltration by IgG4 plasma cells.

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Immunoglobulin G4-Related Disease: An Update

Cited by 21 publications

References 58 publications

Multiple Processes May Involve in the IgG4-RD Pathogenesis: An Integrative Study via Proteomic and Transcriptomic Analysis

Multiple Processes May Involve in the IgG4-RD Pathogenesis: An Integrative Study via Proteomic and Transcriptomic Analysis

A Case of Hypertrophic Pachymeningitis Associated with Immunoglobulin-G4 and c-ANCA

IgG4-assoziierte Orbitopathie als wichtige Differenzialdiagnose eines fortgeschrittenen Silent-Sinus-Syndroms

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