A clinical study of olfactory dysfunction in patients with Mikulicz's disease

Mikulicz's disease (MD) is differentiated from Sjögren's syndrome as an immunoglobulin G4 (IgG4) systemic disease. MD patients often report olfactory dysfunction (OD). To analyze cases of OD associated with MD, we studied clinicopathological and serological findings of MD patients.

A total of 44 MD patients (17 males and 27 females) were examined for OD. We evaluated clinicopathological and serological findings of these patients by dividing them into OD(+) and OD(-) groups.

The mean IgG4 concentration (SD) in such cases was 950.5 (797.5)mg/dl. Of the 44 patients, 20 (45%) had OD even though no abnormalities, such as obstructive and inflammatory disease, were detected in their nasal cavities and sinuses. The two groups did not show significant differences in background characteristics, such as age, sex, IgG4 concentration, presence or absence of allergic rhinitis, and presence or absence of extrasalivary gland lesions. We found abundant IgG4-positive plasmacytes in the nasal mucosa specimens of the OD(+) group but not in that of the OD(-) group.

Nasal mucosa in the MD patients with OD was infiltrated with IgG4-positive plasmacytes. We concluded that OD may be associated with infiltration by IgG4-positive cells.