Diagnostic criteria for IgG4-related ophthalmic disease
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Immunoglobulin G4 (IgG4)-related disease is a novel clinical entity characterized by infiltration of IgG4-immunopositive plasmacytes and elevated serum IgG4 concentration accompanied by enlargement of and masses in various organs, including the lacrimal gland, salivary gland, and pancreas. Recent studies have clarified that conditions previously diagnosed as Mikulicz disease as well as various types of lymphoplasmacytic infiltrative disorders of the ocular adnexa are consistent with a diagnosis of IgG4-related disease. Against this background, the diagnostic criteria for IgG4-related ophthalmic disease have recently been established, based on both the clinical and the histopathologic features of the ocular lesions. This article reviews these new criteria with reference to the comprehensive diagnostic criteria for IgG4-related disease for all systemic conditions reported in 2012.
KeywordsIgG4 Plasma cell Lacrimal gland Mikulicz disease
This work was supported by the Research Program for Intractable Disease (IgG4-related disease), Health and Labor Sciences Research Group of the Ministry of Health, Labor, and Welfare of Japan.
Conflicts of interest
H. Goto, None; M. Takahira, None; A. Azumi, None.
- 4.Mikulicz J. Über eine eigenartige symmetrische Erkrankung der Tränen- und Mundspeicheldrüsen. Beitr Z Chir Festschr Theodor Billroth. 1892;2:610–30.Google Scholar
- 6.Yamada K, Kawano M, Inoue R, Hamano R, Kakuchi Y, Fujii H, et al. Clonal relationship between infiltrating immunoglobulin G4 (IgG4)-positive plasma cells in lacrimal glands and circulating IgG4-positive lymphocytes in Mikulicz’s disease. Clin Exp Immunol. 2008;152:432–9.CrossRefPubMedCentralPubMedGoogle Scholar