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. Author manuscript; available in PMC: 2026 Oct 1.
Published in final edited form as: Arch Intern Med Res. 2026 Sep 10;9(3):325–330. doi: 10.26502/aimr.0259

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