Table 1.

Diagnostic criteria of IgG4+ Mikulicz’s disease (Japanese Sjögren’s Syndrome Society, 2008). Differential diagnosis is necessary from other distinct disorders, including sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, lymphoma, and cancer. The diagnostic criteria for Sjögren’s syndrome (SS) may also include some patients with IgG4+ Mikulicz’s disease; however, the clinicopathological conditions of patients with typical SS and IgG4+ Mikulicz’s disease are different.

1. Symmetrical swelling of at least 2 pairs of the lacrimal, parotid, or 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.