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Table 1 Diagnostic criteria for IgG4-related ophthalmic disease15

From: IgG4-related disease in patients with idiopathic orbital inflammation

Diagnostic criteria for IgG4-related ophthalmic disease

1. Imaging studies show enlargement of the lacrimal gland, trigeminal nerve, or extraocular muscle, as well as masses, enlargement, or hypertrophic lesions in various ophthalmic tissues.

2. Histopathological examination shows marked lymphocyte and plasmacyte infiltration and sometimes fibrosis. A germinal center is frequently observed. IgG4 + plasmacytes are found and satisfy the following criteria: ratio of IgG4 + cells to IgG + cells of 40% or above, or > 50 IgG4 + cells per high-power field (× 400).

3. Blood test shows elevated serum IgG4 (> 1.35 g/L).

Definite: 1 + 2 + 3, Probable: 1 + 2, Possible: 1 + 3.