Fcγ-receptor 3B ( FCGR3B ) copy number variations in patients with eosinophilic granulomatosis with polyangiitis

Martorana, Davide; Bonatti, Francesco; Alberici, Federico; Gioffredi, Andrea; Reina, Michele; Urban, Maria Letizia; Maritati, Federica; Adorni, Alessia; Radice, Antonella; Pizzolato, Silvia; Gregorini, Gina; Jeannin, Guido; Guida, Giuseppe; Boita, Monica; Pesci, Alberto; Moroni, Gabriella; Neri, Tauro Maria; Sinico, Renato Alberto; Vaglio, Augusto

doi:10.1016/j.jaci.2015.09.053

Cited by 17 publications

(10 citation statements)

References 12 publications

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“…The Fcγ receptor 3B (FCGR3B) is mainly expressed on neutrophils and contributes to the clearance of immune complexes by neutrophils. MARTORANA et al [125] demonstrated that FCGR3B deficiency predisposes to EGPA and is particularly associated with vasculitis on biopsy (OR 3.23, 95% CI 1.3-8.02; p=0.008).…”

Section: Talc-induced Granulomatosismentioning

confidence: 99%

Differential diagnosis of granulomatous lung disease: clues and pitfalls

et al. 2017

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Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis.

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Section: Talc-induced Granulomatosismentioning

confidence: 99%

Differential diagnosis of granulomatous lung disease: clues and pitfalls

et al. 2017

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“…Genetic variants may also be shared as predisposing factors by the different AAV forms; this is the case not only of single nucleotide polymorphisms but also of gene copy number variations (CNVs); for example, FcGR3B gene CNVs, which are linked to several autoimmune disorders, have been associated with EGPA in a recent study 35 and also with GPA and MPA in an earlier study. 36 These and other variants may constitute a common genetic background for AAV.…”

Section: Disease Assessmentmentioning

confidence: 99%

The European Vasculitis Society 2016 Meeting Report

Bajema

Bruijn

Casian

et al. 2017

Kidney International Reports

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The 2016 European Vasculitis Society (EUVAS) meeting, held in Leiden, the Netherlands, was centered around phenotypic subtyping in antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV). There were parallel meetings of the EUVAS petals, which here report on disease assessment; database; and long-term follow-up, registries, genetics, histology, biomarker studies, and clinical trials. Studies currently conducted will improve our ability to discriminate between different forms of vasculitis. In a project that involves the 10-year follow-up of AAV patients, we are working on retrieving data on patient and renal survival, relapse rate, the cumulative incidence of malignancies, and comorbidities. Across Europe, several vasculitis registries were developed covering over 10,000 registered patients. In the near future, these registries will facilitate clinical research in AAV on a scale hitherto unknown. Current studies on the genetic background of AAV will explore the potential prognostic significance of genetic markers and further refine genetic associations with distinct disease subsets. The histopathological classification of ANCA-associated glomerulonephritis is currently evaluated in light of data coming out of a large international validation study. In our continuous search for biomarkers to predict clinical outcome, promising new markers are important subjects of current research. Over the last 2 decades, a host of clinical trials have provided evidence for refinement of therapeutic regimens. We give an overview of clinical trials currently under development, and consider refractory vasculitis in detail. The goal of EUVAS is to stimulate ongoing research in clinical, serological, and histological management and techniques for patients with systemic vasculitis, with an outlook on the applicability for clinical trials.

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“…The genetic basis of eosinophilic granulomatosis with polyangiitis (EGPA; formerly Churg-Strauss syndrome) was investigated, focusing on copy number variations involving Fcg receptor (FcgR) genes because low copy number variations have been associated with other autoimmune diseases. 81 Fc fragment of IgG receptor 3B (FCGR3B) deficiency (ie, low copy number) was shown to be overrepresented in patients with EGPA, suggesting that this can affect immunoglobulin clearance or perturb the balance between inhibitory and activation Fc receptors, especially because EGPA is characterized by increased autoantibody and IgG 4 levels. 81…”

Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

“…81 Fc fragment of IgG receptor 3B (FCGR3B) deficiency (ie, low copy number) was shown to be overrepresented in patients with EGPA, suggesting that this can affect immunoglobulin clearance or perturb the balance between inhibitory and activation Fc receptors, especially because EGPA is characterized by increased autoantibody and IgG 4 levels. 81…”

Section: Eosinophilic Granulomatosis With Polyangiitismentioning

confidence: 99%

Advances in mechanisms of allergic disease in 2016

Rothenberg¹,

Saito²,

Peebles³

2017

Journal of Allergy and Clinical Immunology

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This review highlights advances in mechanisms of allergic disease, particularly type 2 innate lymphoid cells; T2 lymphocytes; eicosanoid regulation of inflammation; extracellular vesicles in allergic responses; IL-33; microbiome properties, especially as they relate to mucosal barrier function; and a series of findings concerning the allergic inflammatory cells eosinophils, basophils, and mast cells. During the last year, mechanistic advances occurred in understanding type 2 innate lymphoid cells, particularly related to their response to ozone, involvement with experimental food allergy responses, and regulation by IL-33. Novel ways of regulating T2 cells through epigenetic regulation of GATA-3 through sirtuin-1, a class III histone deacetylase, were published. The understanding of eicosanoid regulation of inflammation increased and focused on additional properties of phospholipase A and the role of prostaglandin D and its receptors and inhibitory prostaglandin E pathways. Mechanisms through which extracellular vesicles are released and contribute to allergic responses were reported. There was a deeper appreciation of mucosal barrier function, the epithelial alarmin IL-33, and the microbiome. Finally, there were advances concerning allergic inflammatory cells (mast cells, basophils, and eosinophils) that will undoubtedly have an effect on disease understanding and new therapeutic strategies.

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Fcγ-receptor 3B ( FCGR3B ) copy number variations in patients with eosinophilic granulomatosis with polyangiitis

Cited by 17 publications

References 12 publications

Differential diagnosis of granulomatous lung disease: clues and pitfalls

Differential diagnosis of granulomatous lung disease: clues and pitfalls

The European Vasculitis Society 2016 Meeting Report

Advances in mechanisms of allergic disease in 2016

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