Evaluation and Clinical Validity of a New Questionnaire for Mikulicz's Disease

Table 1

Characteristics of analyzed patients with Mikulicz’s disease.

(1) Persistent (>3 months), symmetrical swelling of the lacrimal, parotid, and submandibular glands, involving at least two pairs.
(2) Serologically high levels of immunoglobulin (lg)G4 (≥13.5 mg/L).
(3) Marked lgG4-positive plasmacyte infiltration (≥50% lgG4-positive/lgG-positive cells in five high power fields) into lacrimal and salivary gland tissues.

In terms of diagnosis, lgG4-related Mikulicz’s disease is defined as satisfying item 1 and either item 2 and/or item 3. This form of systemic lgG4-related disease often accompanies multiple organ lesions. Sarcoidosis, Castleman’s disease, Wegener’s granulomatosis, and malignant lymphoma need to be considered as differential diagnoses.