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Sjogren's syndrome

Sjögren's syndrome is an autoimmune disorder in which immune cells attack and destroy the 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. more...

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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.

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 test measures 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 examination is 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 biopsy can reveal lymphocytes clustered 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, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes (some patients with more severe problems use goggles to increase local humidity or 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 drugs may be used to treat musculoskeletal symptoms. For individuals with severe complications, corticosteroids or immunosuppressive drugs may be prescribed. Also, disease-modifying antirheumatic drugs (DMARDs) such as methotrexate may be helpful.

Read more at Wikipedia.org


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Myths & facts...about Sjogren's syndrome
From Nursing, 6/1/02 by McConnell, Edwina A

MYTH: Sjogren's syndrome is an uncommon auto-- immune disease.

FACT: A debilitating chronic rheumatic disease, Sjbgren's syndrome is the most common autoimmune disease affecting women. From 1 to 2 million Americans have the disease; 95% are women and most are elderly. It may occur as a primary disease (primary Sjogren's syndrome) or in association with other rheumatic disorders (secondary Sjogren's syndrome).

MYTH: Initially, Sjogren's syndrome causes gastrointestinal symptoms.

FACT: The first symptoms of Sjogren's syndrome typically are xerostomia (dry mouth) and xerophthalmia (dry eyes). These symptoms develop when immune cells infiltrate the salivary and lacrimal glands, causing a progressive decline in sight, smell, and taste. As the disease progresses, immune cells infiltrate other tissues and organs, causing various nonglandular signs and symptoms.

MYTH: The most common nonglandular effect of Sjogren's syndrome is chronic obstructive pulmonary disease.

FACT: Rheumatoid arthritis is the most common non-- glandular manifestation of Sjogren's syndrome.

MYTH: The onset of disease is dramatic.

FACT: Like other autoimmune diseases, Sjogreres syndrome is insidious in onset. The patient may start having symptoms between the ages of 35 and 50, but she may not be diagnosed for another 10 years because the disorder progresses so slowly. By the time she's diagnosed, she may already have suffered organ damage. MYTH: The goal of treatment is cure.

FACT: Sjogren's syndrome is incurable. The goals of treatment are to manage symptoms and to prevent or minimize organ damage.

MYTH: Sjogren's syndrome is mostly a nuisance that doesn't require any lifestyle changes.

FACT: Someone with Sjogen's syndrome suffers from sleep disturbances and fatigue, which may require lifestyle changes. Other symptoms decrease the acuity of senses essential for physiologic functioning and emotional responsiveness, which can seriously diminish her quality of life.

BY EDWINA A. McCONNELL, RN, PHD, FRCNA

Edwina A. McConnell is an independent nurse-consultant in Gorham, Me. Selected references for this article are available on request.

Copyright Springhouse Corporation Jun 2002
Provided by ProQuest Information and Learning Company. All rights Reserved

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