Table 2.

Diagnostic criteria for Immunoglobulin G4-related ophthalmic disease (IgG4-ROD), 2014

(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) Blood test shows elevated serum IgG4 (≧135 mg/dl)
(3) Histopathologic examination shows:
A. Marked lymphocyte and plasmacyte infiltration, and sometimes fibrosis
B. A germinal center is frequently observed
C. Infiltration of IgG4+ plasma cells: ratio of lgG4+/IgG+ cells ≧40 % or ≧50 IgG4+ plasma cells per high power field
Definite	IgG4-ROD:	(1) + (2) + (3)
Probable	IgG4-ROD:	(1) + (3)
Possible	IgG4-ROD:	(1) + (2)

It is important to differentiate IgG4-ROD from malignant tumors (e.g., cancer, lymphoma) and from similar diseases (e.g., Sjögren’s syndrome, primary sclerosing cholangitis, Castleman disease, secondary retroperitoneal fibrosis, Wegener granulomatosis, sarcoidosis, Churg-Strauss syndrome) by conducting additional histopathologic examination