T Cell Roles and Activity in Chronic Sclerosing Sialadenitis as IgG4-Related Disease: Current Concepts in Immunopathogenesis
Table 1
The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-related disease (IgG4-RD) proposed by Umehara et al. [31].
1. Clinical and radiological features
One or more organs show diffuse or localized swelling or a mass or nodule characteristic of IgG4-RD. In single organ involvement, lymph node swelling is omitted.
2. Serological diagnosis
Serum IgG4 levels greater than 135 mg/dl.
3. Pathological diagnosis
Positivity for two of the three following criteria:
1. Dense lymphocyte and plasma cell infiltration with fibrosis.
2. Ratio of IgG4+ plasma cells/IgG+ cells greater than 40% and the number of IgG4+ plasma cells greater than 10 per high powered field
3. Typical tissue fibrosis, particularly storiform fibrosis, or obliterative phlebitis
