Table 2.

The Three Major Histopathological Features Associated with IgG4-Related Disease and the Minimal Criteria in a New Organ/Site in the International Pathological Consensus¹⁸

The three major histopathological features associated with IgG4-RD Dense lymphoplasmacytic infiltrate Fibrosis, arranged at least focally in a storiform pattern Obliterative phlebitis Other histopathological features associated with IgG4-RD are: Phlebitis without obliteration of the lumen Increased numbers of eosinophils Minimal criteria for IgG4-RD in a new organ/site Characteristic histopathological findings with an elevated IgG4t plasma cells and IgG4-to-IgG ratio High serum IgG4 concentrations Effective response to glucocorticoid therapy Reports of other organ involvement that is consistent with IgG4-RD

IgG4-RD, IgG4-related disease.