Table 1.

Comprehensive diagnostic criteria for IgG4-RD

1	Clinical examination: clinical history, physical examination, imaging
2	Immunological examination: IgG4 in serum >135 mg/dL or elevated IgG4/IgG ratio; possibly accompanied by other laboratory tests, such as IgE, γ-globulin, or a complement
3	Histopathologic examination: lymphoplasmocytic infiltration with storiform fibrosis and obliterative phlebitis, infiltration by IgG4-positive plasma cells (IgG4+/IgG+ >40%)

1 + 2 = possible IgG4-RD; 1 + 3 = probable IgG4-RD; 1 + 2 + 3 = definite IgG4-RD. Adapted from Umehara et al. [21].