Sjögren's syndrome

Sjögren's syndrome is an autoimmune disorderin which immune cellsattack and destroy the glandsthat produce tearsand saliva. It is named after Swedish ophthalmologistHenrik Sjögren(1899-1986), who first described it. Sjögren's syndrome is also associated with rheumaticdisorders such as rheumatoid arthritis, and it is rheumatoid factorpositive in 90 percent of cases. The hallmark symptoms of the disorder are dry mouthand dry eyes. In addition, Sjögren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organsof 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.

Diagnosis

Diagnosing Sjögren?s syndrome is complicated by the range of symptoms a patient may manifest, and the similarity between symptoms from Sjögren's syndrome and those caused by other conditions. Nevertheless, several tests can confirm a diagnosis of Sjögren's syndrome.

Blood tests can be done to determine if a patient has high levels of antibodies that are indicative of the condition, such as anti-nuclear antibody(ANA) and rheumatoid factor, which are associated with autoimmune diseases. Typical Sjögren syndrome ANA patterns are SSA/Ro and SSB/La, of which SSB/La is far more specific; SSA/Ro is associcated with numerous other autoimmune conditions but are often present in Sjögren's (Franceschini & Cavazzana I 2005).

The Schirmer testmeasures the production of tears: a strip of filter paper is held inside the lower eyelid for five minutes, and its wetness is then measured with a ruler. A slit-lamp examinationis done to look for dryness on the surface of the eye. Salivary gland function can be tested by collecting saliva and determining the amount produced. A lip biopsycan reveal lymphocytesclustered around salivary glands, and damage to these glands due to inflammation.

Treatment

There is neither a known cure for Sjögren's syndrome nor a specific treatment to permanently restore gland secretion. Instead, treatmentis generally symptomatic and supportive. Moisture replacement therapies such as artificial tearsmay ease the symptoms of dry eyes (some patients with more severe problems use gogglesto increase local humidityor have punctal plugs inserted to help retain tears on the occular surface for a longer time). Additionally, Cyclosporine(Restasis®) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion. Prescription drugs are also available that help to stimulate salivary flow, such as cevimeline (Evoxac™) and pilocarpine(Salagen™). Nonsteroidal anti-inflammatory drugsmay be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroidsor immunosuppressive drugs may be prescribed. Also, disease-modifying antirheumatic drugs(DMARDs) such as methotrexatemay be helpful.

Prognosis

Sjögren's can damage vital organs of the body with symptoms that may plateau, worsen, or go into remission. Some people may experience only the mild symptoms of dry eyes and mouth, while others go through cycles of good health followed by severe disease. Many patients are able to treat problems symptomatically. Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating. Debilitating fatigueand joint paincan seriously impair quality of life.

Epidemiology

Sjogren's syndrome affects 1-4 million people in the United States. Most people are more than 40 years old at the time of diagnosis. Women are 9 times more likely to have Sjogren's syndrome than men.

Research

The goals of research on disorders such as Sjögren's syndrome focus on increasing knowledge and understanding of the disorder, improving diagnostic techniques, and finding ways to treat, prevent, and cure the disorder.

See also

• Benign lymphoepithelial lesion
• Keratoconjunctivitis sicca
• Xerostomia

References

• Franceschini F, Cavazzana I. Anti-Ro/SSA and La/SSB antibodies. Autoimmunity 2005;38:55-63. PMID 15804706.
• Sjögren H. Zur Kenntnis der keratoconjunctivitis sicca. Doctoral thesis, 1933.

External links

• The original text from this article was obtained from a public domainresource at: www.ninds.nih.gov/health_and_medical/disorders/sjogrens_doc
• Sjögren's Syndrome Foundation
• Sjögren's Syndrome article by Mayo Clinic staffde:Sjögren-Syndrom

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It uses material from the http://en.wikipedia.org/wiki/Sj%C3%B6gren%27s+syndrome Wikipedia article Sjögren's syndrome.