Sjögrens Syndrome
In 1933, Henrick Sjögren reported the association of dry eyes (xerophthalmia) and dry mouth (xerostomia), with polyarthritis. The presence of sicca (dryness) symptoms in the absence of another connective tissue disease is designated "primary Sjögrens syndrome", whereas their occurrence in association with another autoimmune process, such as rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, or polymyositis, is termed secondary Sjogrens syndrome.
It has been estimated that Sjögrens syndrome (SS) affects 1:50 to 1:1000 people in the United States. Much of the difference in reported prevalence is likely due to referral bias at academic health centers and lack of agreement upon diagnostic criteria. Approximately 90% of patients are female, and men generally manifest milder disease. The disease most commonly develops in the fourth and fifth decades of life, but has been reported in all ages.
The etiology of SS remains an enigma. However, detection of antinuclear antibodies and rheumatoid factor reflects the autoimmune nature of the process. Serologic studies have also demonstrated the frequent occurrence of anti-Ro (SS-A) antibodies and, to a lesser extent, anti-La (SS-B) antibodies. Expression of these antibodies is associated with extra-glandular manifestations of SS, including serologic hyper-reactivity (e.g. hypergammaglobulinemia), hypocomplementemia, cytopenias (e.g. anemia, leukopenia, and thrombocytopenia), lymphadenopathy, and cutaneous vasculitis.
Sjögrens syndrome is marked by lymphocytic infiltration of the lacrimal and salivary glands. Extra-glandular manifestations are common and result from similar infiltration of various organs. Immunohistologic studies have shown that the lymphocytic infiltrate is comprised predominantly of CD4-positive T cells, primarily of the Th1-type. Unlike normal epithelium, the glandular epithelial cells express MHC class II antigens and B7 co-stimulatory molecules, allowing them to serve as antigen-presenting cells.
Fox RI, Michelson P, and Tornwall J. Sjögren's Syndrome in Textbook of Rheumatology, Kelley WN, Harris ED, Ruddy S, Sledge, CB. (eds) W.B. Saunders, Co., Philadelphia. pgs. 1027-1038, 2001.
Fox RI. Clinical features, pathogenesis and treatment of Sjögren's syndrome. Curr Opin Rheumatol 8:438-445, 1996.
Fox RI. Treatment of the patient with Sjögren's Syndrome. Rheum Dis Clin North Am 18:699-709, 1992.
For additional patient information, contact:
The National Sjögren's Syndrome Association
3201 West Evans Drive
Phoenix, AZ 85023
(800) 395-6772
The Sjögren's Syndrome Foundation, Inc
8120 Woodmont Ave.
Suite 530
Bethesda, MD 20814
(301) 718-0300