Table 1.
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The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD.2

  • 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 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

Definite Diagnosis: 1 + 2 + 3; Probable Diagnosis: 1 + 3; Possible Diagnosis: 1 + 2.

  • 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 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

Definite Diagnosis: 1 + 2 + 3; Probable Diagnosis: 1 + 3; Possible Diagnosis: 1 + 2.
Table 1.
Open in new tab

The 2020 revised comprehensive diagnostic (RCD) criteria for IgG4-RD.2

  • 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 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

Definite Diagnosis: 1 + 2 + 3; Probable Diagnosis: 1 + 3; Possible Diagnosis: 1 + 2.

  • 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 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

Definite Diagnosis: 1 + 2 + 3; Probable Diagnosis: 1 + 3; Possible Diagnosis: 1 + 2.
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