Mikulicz's disease and Sjögren's syndrome

Invest Ophthalmol Vis Sci. 2000 Jun;41(7):1666-73.

Abstract

Purpose: To characterize lacrimal gland function and lymphocyte infiltration in patients with Mikulicz's disease (MD) and Sjögren's syndrome (SS).

Methods: Four patients with MD and 5 with SS were recruited, on whom were performed Schirmer test I (Schirmer test without anesthesia), Schirmer test with nasal stimulation, and vital staining of the ocular surface. The lacrimal gland was then biopsied and the tissues stained with CD3, CD4, CD8, B220, APO2.7, Fas, and Fas ligand (Fas-L) antibodies.

Results: Although regular Schirmer test results in the MD group were less than 10 mm, those with nasal stimulation, 38.1 +/- 3.4 mm, were significantly greater than the SS group. There were minimal ocular surface changes in MD. Morphologic staining with hematoxylin and eosin was identical in both groups, but the acinar cells were stained with APO2.7 only in the SS group. There was strong Fas and Fas-L staining in SS patients but not in those with MD.

Conclusions: Lacrimal gland acinar cells in those with MD maintained their function and were not programmed for cell death. The sicca syndrome was not observed in MD patients. Although the pathology is similar for MD and SS, the difference in acinar cell apoptosis and function can explain clinical differences.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • CD3 Complex / metabolism
  • CD4 Antigens / metabolism
  • CD8 Antigens / metabolism
  • Fas Ligand Protein
  • Female
  • Humans
  • Immunoenzyme Techniques
  • Lacrimal Apparatus / metabolism
  • Lymphocytes / metabolism
  • Lymphocytes / pathology
  • Male
  • Membrane Glycoproteins / metabolism
  • Middle Aged
  • Mikulicz' Disease / metabolism
  • Mikulicz' Disease / pathology*
  • Sjogren's Syndrome / metabolism
  • Sjogren's Syndrome / pathology*
  • Tears / metabolism
  • fas Receptor / metabolism

Substances

  • CD3 Complex
  • CD4 Antigens
  • CD8 Antigens
  • FASLG protein, human
  • Fas Ligand Protein
  • Membrane Glycoproteins
  • fas Receptor