Table 1:
Summary of the 2020 Revised Comprehensive Diagnostic (RCD) Criteria for IgG4-Related Diseases.
| Detailed Diagnostic (RCD) Criteria for IgG4-Related Diseases | |
|---|---|
| 1. Clinical and radiological feature | One or more organs show diffuse or localized swelling, mass, nodule characteristic for IgG4-related disease. In single organ involvement, lymph node swelling is omitted. |
| 2. Serological diagnosis | Serum IgG4 levels are more than 135 mg/dL. |
| 3. Pathological diagnosis | Positivity for two of the following three criteria: |
| a. Dense lymphocyte and plasma cell infiltration with fibrosis. | |
| b. Ratio of IgG4-positive plasma cells/IgG-positive cells greater than 40% and the number of IgG4-positive plasma cells greater than 10 per high-powered field | |
| c. Typical tissue fibrosis, particularly storiform fibrosis, or obliterative phlebitis* | |
| Diagnostic Classification | Definite: if +ve clinical and radiological features, +ve serological diagnosis, and +ve pathological diagnosis |
| Probable: if +ve clinical and radiological features and +ve pathological diagnosis | |
| Possible: if +ve clinical and radiological features and +ve serological diagnosis | |