Clinical and pathological differences between mikulicz's disease and Sjögren's syndrome

• Published in: Rheumatology (Oxford, England) England), 2005-02, Vol.44 (2), p.227-234
• Main Authors: YAMAMOTO, M, HARADA, S, OHARA, M, SUZUKI, C, NAISHIRO, Y, YAMAMOTO, H, TAKAHASHI, H, IMAI, K
• Format: Article
• Language: English
• Subjects: Aged; Biological and medical sciences; Female; Glucocorticoids - therapeutic use; Humans; Immunoglobulin G - blood; Immunohistochemistry - methods; Interferon-gamma - blood; Interleukin-4 - blood; Lacrimal Apparatus - pathology; Male; Medical sciences; Middle Aged; Mikulicz' Disease - drug therapy; Mikulicz' Disease - immunology; Mikulicz' Disease - pathology; Salivary Glands - pathology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Sjogren's Syndrome - drug therapy; Sjogren's Syndrome - immunology; Sjogren's Syndrome - pathology
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/keh447

Mikulicz's disease (MD) has been included within the diagnosis of primary Sjögren's syndrome (SS), but represents a unique condition involving enlargement of the lachrymal and salivary glands and characterized by few autoimmune reactions and good responsiveness to glucocorticoids. We have previously described elevated immunoglobulin (Ig) G4 in the serum of four patients with MD. In this paper, we accumulated more MD cases and undertook clinical and histopathological analysis of these patients to clarify differences between MD and SS. We diagnosed seven patients with MD according to the following criteria: (i) visual confirmation of symmetrical and persistent swelling in more than two lachrymal and major salivary glands; (ii) prominent mononuclear infiltration of lachrymal and salivary glands; and (iii) exclusion of other diseases that present with glandular swelling, such as sarcoidosis and lymphoproliferative disease. We summarized the clinical and serological characteristics (IgG subclasses and IFN-gamma/IL-4 ratio) of seven patients with MD, compared with SS with glandular swelling (SSw) and without glandular swelling (SSo). After steroid administration, we analysed changes in IgG subclasses in MD. Labial salivary gland specimens in MD, SSw and SSo were stained with anti-IgG4 antibodies. The concentration (+/-s.d.) of IgG4 was 1169.7 +/- 892.2 mg/dl in MD, 24.4 +/- 7.0 mg/dl in SSw (P