Sjögren's syndrome overview
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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.