Table 2 Proposed diagnostic criteria for IgG4-RD according to ‘Comprehensive diagnostic criteria for IgG4-related disease’ [2, 14, 15]

Histology

(i)The specimen pathology shows the dense lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis, the infiltration of IgG4-positive cells, and IgG cells more than IgG4 cells ratio of 40%;

Imaging

(ii)clinical/radiological examination showing characteristic diffuse or localized swelling or masses in single or multiple organs;

Serology

(iii) serum IgG4 concentration > 135 mg/dL;

(iv)Inflammatory markers such as white blood cells count and C-reactive protein concentrations are not elevated, despite the degree of lesions, their spread on imaging analysis and massive cellular infiltration on pathological examination;

Other organ involvement

(v)Characteristic findings of IgG4-RD in other organs, including autoimmune pancreatitis, lung involvement, et al.

Treatment

(vi)response to steroids.