Diagnostic criteria for IgG4-related ophthalmic disease, based on the criteria established in 2014 by the Japanese Ophthalmological Society [84].
(A) Imaging studies: enlargement of the lacrimal gland, trigeminal nerve, or extraocular muscle; masses, enlargement, or hypertrophic lesions in various ophthalmic tissues (B) Histopathologic examination: marked lymphocytic and plasmacytic infiltration, sometimes fibrosis. Often the presence of a germinal center. IgG4+ plasmacytes are found and meet the following criteria: a ratio of IgG4+ cells to IgG+ cells of 40% or above or more than 50 IgG4+ cells per HPF (×400) (C) Blood test: elevated serum IgG4 concentration (>135 mg/dl)
Based on the fulfillment of the criteria mentioned above, the probability of the diagnosis is defined as follows: “Definitive diagnosis”—when criteria A, B, and C are fulfilled “Probable diagnosis”—when A and B are fulfilled “Possible diagnosis”—when A and C are fulfilled
