Sjögren's syndrome (SS) is a progressive autoimmune rheumatic disorder.1-6 Its precise etiology is unknown, although several contributing factors have been identified. One theory is that the condition results from complications related to infection with the Epstein-Barr virus.4 Primary exposure to or reactivation of Epstein-Barr virus elicits expression of the human leukocyte antigen complex. This is recognized by T lymphocytes (CD 4+) resulting in the release of cytokines (tumor necrosis factor, interleukin-2, interferon-gamma, and others). A genetic marker specific for Sjögren's syndrome, HLA-DR4, has been identified.1,2 According to the World Health Organization, the prevalence of Sjögren 's syndrome is unknown.7 A recent epidemiologic study in Sweden estimated the prevalence in the adult population to be 2.7%.8 In the United States, 10 years ago, the number of patients with Sjögren's syndrome was thought to be fewer than 100,000.9 This number today is estimated to be more than 1 million.10 Sjögren's syndrome has been reported in nearly every major country of the world, and the geographic distribution of cases appears to be relatively uniform.11 Sjögren's syndrome typically affects women (90%) during the fourth or fifth decade of life.12 Isolated cases of Sjögren's syndrome in children have been reported.13.
|Original language||English (US)|
|Number of pages||11|
|State||Published - Oct 1 1999|