Beneficial effects of steroid therapy for Mikulicz's disease.

Beneficial effects of steroid therapy for Mikulicz's disease SIR, Mikulicz's disease (MD) represents a unique condition involving symmetrical enlargement of the lacrimal and salivary glands, the cause of which is unknown. It has been included within the diagnostic criteria for primary Sjo¨gren's syndrome (SS) due to their histological resemblance [1]. However, it is clear that the pathogenesis of MD differs from that of SS because an elevated serum IgG4 concentration and infiltration of abundant plas-macytes with IgG4 into the lacrimal and salivary glands are seen in MD patients [2]. Clinically, MD exhibits good responsiveness to glucocorticoids, showing improvements in not only lacrimal and salivary gland swelling but also secretion. We attempted to objectively document this phenomenon and to serologically evaluate steroid efficacy. Analyses were performed in eight MD patients (two men, six women) who consulted doctors at Sapporo Medical University between April 1997 and January 2005. MD was diagnosed according to the following criteria: (i) persistent (more than 3 months) symmetrical swelling of more than two lacrimal and major salivary glands; (ii) prominent mononuclear infiltration of lacrimal and salivary glands; and (iii) exclusion of other diseases that exhibit glandular swelling, such as sarcoidosis or lymphopro-liferative disease [2]. Lacrimal gland function was evaluated by Schirmer's test, and salivary gland function was examined by Saxon's test. IgG subclass was measured by nephelometry. These tests were performed before and after treatment. Mann–Whitney's U-test was used for data comparison, and values of P<0.05 were considered statistically significant. After prescription of 30–40 mg prednisolone/day, lacrimal secretion increased from 6. Glucocorticoids have long been known to effectively improve lacrimal and salivary gland swelling in MD. However, we noted that sicca symptoms were improved when we followed MD patients being treated with steroids. The present results confirmed a tendency for improvement in lacrimal and salivary secretion 2–3 months from the start of steroid treatment in MD. On the other hand, in SS, steroid therapy makes the lacrimal and salivary glands smaller, but is not able to improve gland secretion. SS is an autoimmune disease that mainly destroys glands. We initially hypothesized that steroids are not effective in SS because it is diagnosed after some degree of progression, while steroids are effective in MD because it is diagnosed earlier (onset of changes in facial appearance). However, our MD cases suffered from dryness for several years, and yet gland function improved after gluco-corticoid administration. Tsubota reported fewer apoptotic cells …