Sjögren's syndrome overview: Difference between revisions
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
The [[incidence]] of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The [[prevalence]] of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California. | |||
==Risk Factors== | ==Risk Factors== | ||
Revision as of 16:34, 10 April 2018
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Sjögren's syndrome Microchapters |
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Sjögren's syndrome overview On the Web |
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American Roentgen Ray Society Images of Sjögren's syndrome overview |
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Risk calculators and risk factors for Sjögren's syndrome overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the exocrine glands that produce tears and saliva. It is named after Swedish ophthalmologist Henrik Sjögren (1899-1986), who first described it. Sjögren's syndrome is also associated with rheumatic disorders such as rheumatoid arthritis, and it is rheumatoid factor positive in 90 percent of cases. The hallmark symptoms of the disorder are dry mouth and dry eyes (part of what are known as sicca symptoms). In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body, including the kidneys, blood vessels, lungs, liver, pancreas, and brain. Nine out of ten Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.
The primary form, often called sicca syndrome, involves both Keratoconjunctivitis sicca and Xerostomia. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis.
Historical Perspective
In 1892, Johann von Mikulicz-Radecki was to first to describe a patient with with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1933, Henrik Sjögren was the first to describe 19 females with clinical and pathological manifestations of the Sjögren's syndrome.
Classification
American-European Consensus Group(AECG) and American College of Rheumatology (ACR) established the criteria for Sjögren’s syndrome in 2002 and 2012 according to clinical findings.
Pathophysiology
Causes
Common causes of Sjögren's syndrome include viral infection such as Epstein-Barr virus (EBV), Coxsackie virus, Hepatitis C virus, Cytomegalovirus (CMV), Human herpesvirus 6 (HHV-6), Retroviruses and genetic factors.
Differentiating Sjögren's syndrome overview from Other Diseases
Epidemiology and Demographics
The incidence of Sjögren's syndrome is approximately 4 per 100,000 individuals worldwide. The prevalence of Sjögren's syndrome is approximately 43 per 100,000 individuals worldwide. Female are more commonly affected by Sjögren's syndrome than male. The majority of Sjögren's syndrome cases are reported in China, Japan, and California.