Orbital immunoglobulin-G4-related disease: case series and literature review.
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Cites background from "Orbital immunoglobulin-G4-related d..."
...Although orbital IgG4-RD is a disease predominantly affecting the adults (mean = 50 years), children in the paediatric age-group may also be affected [21, 22]....
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References
2,355 citations
"Orbital immunoglobulin-G4-related d..." refers background in this paper
...IgG4 is the least abundant subclass of IgG in human serum accounting for 3–6%.(20) It has been associated with many pathologic conditions, most of which have a protective effect and do not show any rise in serum IgG4 titre....
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"Orbital immunoglobulin-G4-related d..." refers background in this paper
...Unlike NSOID, IgG4 may be associated with HLA-DRB1, HLA-DQβ1–57 and autoimmunity.52,53 Unlike IgG4RD, idiopathic myositis and NSOID present with sudden onset of inflammation, pain, redness of eyelids and ocular motility restrictions in addition to proptosis, lid swelling or ptosis.54 In addition, allergic conditions, raised levels of soluble IL-2 receptor and serum IgE hypergammaglobulinaemia have been reported in patients with IgG4-RD.38 Thus, finding of IgG4-positive plasma cells in inflammatory lesions of the orbit warrant the screening of patients for possible presence of systemic diseases....
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...At least two of these are required for the diagnosis of IgG4-RD.(25) Lacrimal glands are exceptions to this rule where the latter two can be inconspicuous or absent....
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...IgG4-RD is characterized by a typical histopathological appearance and an elevated number of IgG4positive plasma cells within the tissue.(25) Three major features associated with IgG4-RD are dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis....
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...IgG4-RD is characterized by a typical histopathological appearance and an elevated number of IgG4positive plasma cells within the tissue.25 Three major features associated with IgG4-RD are dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis.25 At least two of these are required for the diagnosis of IgG4-RD.25 Lacrimal glands are exceptions to this rule where the latter two can be inconspicuous or absent.25 The lymphoplasmacytic infiltrate is in the form of diffusely distributed small lymphocytes with varying degree of plasma cell infiltration....
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...Three major features associated with IgG4-RD are dense lymphoplasmacytic infiltrate, storiform fibrosis and obliterative phlebitis.(25) At least two of these are required for the diagnosis of IgG4-RD....
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"Orbital immunoglobulin-G4-related d..." refers background in this paper
...Interleukin-10-producing type 2, helper T cells are activated, which in turn triggers expansion of IgG4-producing plasma cells.(50,51) Elevations of serum IgG4 titres in our patients could be due to the increased plasma cells at orbital lesions rather than circulating plasma cells....
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