Comprehensive Criteria for the Diagnosis (CCD) of Umehara et al for IgG4-Related Disease

Comprehensive diagnostic criteria for IgG4-related disease:

(1) (diffuse or localized swelling) OR (single or multiple masses) in one or more organs

(2) elevated serum IgG4 concentration (>= 135 mg/dL or 1.35 g/L)

(3) characteristic histologic findings

(3a) lymphoplasmacytic infiltrate with fibrosis

(3b) (> 10 IgG4 positive plasma cells per high power field) OR (ratio of IgG4 positive plasma cells to all plasma cells > 40%)

where:

• The combination of 2+3 is not scored, which would seem to make the diagnosis possible.

There should not be any other diagnosis that might explain the findings.