Primary Sjögren’s syndrome (pSS) is a systemic autoimmune disease with early and gradually progressive lacrimal and salivary dysfunction.
Secondary SS occurs in association with other autoimmune disorders, the most common of which is rheumatoid arthritis.
Minor salivary glands and lacrimal glands in SS exhibit a particular pattern of periductal lymphocytic infiltration known as focal lymphocytic sialadenitis.
About 90% of patients with SS are women.
Sjögren’s syndrome is very common, with a community prevalence of pSS ranging from 0.1% to 0.6% of all individuals.
The major eye problem in SS is keratoconjunctivitis sicca, leading to xerophthalmia. The principal oral manifestation of SS is decreased salivary gland production, leading to xerostomia and a predilection for dental caries.
Extraglandular manifestations of SS include arthralgias, thyroiditis, renal involvement (leading to renal tubular acidosis), peripheral neuropathy, cutaneous vasculitis, and lymphoma.
The risk of lymphoma in pSS is approximately 5%.
Most patients with SS develop increased circulating polyclonal immunoglobulins and autoantibodies. These autoantibodies include two fairly specific antibodies directed against the Ro (SS-A) and La (SS-B) antigens.
■ Anti-Ro and -La antibodies may be associated with fetal heart block during the pregnancies of women with SS.
KeywordsSalivary Gland Minor Salivary Gland Cutaneous Vasculitis Congenital Heart Block Oral Symptom
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- 19.Sjögren’s Syndrome Foundation. And the survey says... The Moisture Seekers 2006;24:1–3.Google Scholar