Are Classification Criteria for IgG4-RD Now Possible? The Concept of IgG4-Related Disease and Proposal of Comprehensive Diagnostic Criteria in Japan

Table 2

Diagnostic criteria for IgG4+ Mikulicz’s disease [12] (approved by the Japanese Society for Sjögren’s Syndrome, 2008).

(1) Symmetrical swelling of at least 2 pairs of lachrymal, parotid, and submandibular glands continuing for more than 3 months,
(2) elevated serum IgG4 (>135 mg/dL),
or
(3) histopathological features including lymphocyte and IgG4+ plasma cell infiltration (IgG4+ plasma cells/IgG+ plasma cells > 50%) with typical tissue fibrosis or sclerosis.
Differential diagnosis is necessary from other disorders, including sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, lymphoma, and cancer. Although the diagnostic criteria for Sjögren’s syndrome (SS) may also include some patients with IgG4+ Mikulicz’s disease, the clinicopathological conditions of patients with typical SS and IgG4+ Mikulicz’s disease are different.