ISSN:
1432-2307
Keywords:
Labial salivary gland biopsy
;
Gougerot-Sjögren's syndrome
;
Clinico-pathology
;
Histoenzymology
;
Electron microscopy
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Summary 119 cases of Sjögren's syndrome are described. Clinically, the female sex predominates (83% of cases); the average age is 56 years. Isolated sicca syndrome is noted in 31 % of cases; it is more often associated with rheumatoid polyarthritis (64%) or with another collagen disease (5%). In addition, in 5 patients, we have found a malignant lymphoproliferative disease in an accessory or a main salivary gland (4 non-Hodgkin malignant lymphomas, 1 chronic lymphoid leukaemia). In all cases, a biopsy from accessory labial glands or from a main salivary gland, corroborates the diagnosis. Histologically, epi-myoepithelial islets are always lacking in accessory glands. We classified labial gland injuries in three groups based on striated duct distention: first, some tubular ectasia with interstitial lymphoid cells; second, much ectasia associated with many lymphoid cells and the beginning of pericanalar sclerosis; third, intensive ectasia, scarce lymphocytes, severe peri- and intralobular sclerosis and extensive destruction of acini. This grading is positively correlated with that of parotid biopsies and also with abnormalies on numerous parotid sialographic radiograms performed simultaneously. The histoenzymological study demonstrates decreased activity of oxydative enzymes in striated duct epithelia and of ATPases in myoepithelial cells. The ultrastructural study confirms these findings. Even at an early stage of the disease, tubular alterations are observed: epithelial cell degeneration (vacuolated mitochondria, loss of cytoplasmic lateral membrane foldings) and myoepithelial cell damage (in particular disappearance of myofilaments). These findings explain tubular ectasia by the loss of contractile function. Simultaneously, intensive regeneration from the intercalated ducts by young “totipotent” cells, sometimes differentiating into epidermoid, secretory or myoepithelial cells, are observed. Lesions in acinar cells are less marked: conglomeration of secretory granules, some autophagosomes and rarely true necrosis. In the interstitial tissue, amongst lymphocytes, plasmocytes and mastocytes, numerous myoepithelial cells are present: young pseudo-fibroblastic cells with poor ergastoplasm and some myoid organelles (a few myofilaments, dark zones and endocytosis vacuoles on cell membrane; degenerated myoepithelial cells whose scanty dark cytoplasm is filled with lipid droplets). Thus, by analogy with the myoepithelial islets observed in parotid biopsies, the myoepithelial cell damage seems to have a specific signification in Sjögren's disease. Regeneration from the intercalated ducts fails to repair these cells and to restore their physiological contractile function. Furthermore, this leads to extratubular migration of young myoid cells which are responsible for progressive accumulation in the interstitial tissue of membranoid material and later collagen sclerosis.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF02155670
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