[Clinical aspects and types of Sjögren's syndrome].
Sjögren's syndrome (SS) is a systemic as well as an organ-specific autoimmune disease, characterized by destructive lymphocytic infiltration of the salivary and lacrimal glands. We divided SS patients into three stages: stage I is glandular SS, stage II is extraglandular SS, and stage III is extraglandular SS with lymphoid malignancy. The lymphoaggressive nature of the disease appears to lead SS patients from stage I to II and from stage II to III. However, stage III patients made up only 5% of SS patients. Many patients remain stable in stages I or II for as long as 10 or 20 years. Therefore, we can assume that there are factors which trigger patients in stages I or II to progress to stages II or III and that only those patients who have such factors progress from stages I or II to stages II or III, respectively. Accumulation in the salivary glands or in the peripheral blood of B cells which have rearrangement of the RF-related germline gene Vg or over-expression of the bcl-2 gene in the lymphoepithelial lesion might be included among these factors.
Intractable Disease Section, Kanazawa Medical University.
S
S
Sugai
S
S
Takeshita
Y
Y
Ogawa
M
M
Fukutoku
Y
Y
Masaki
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D005260:Female; D006801:Humans; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012469:Salivary Glands / Q000166:cytology; D012859:Sjogren's Syndrome / Q000145:classification* / Q000473:pathology / Q000503:physiopathology