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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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Treating Dryness Associated with Sjogren's Syndrome - Statistical Data Included
From American Family Physician, 7/1/99

Sjogren's syndrome is a chronic autoimmune rheumatic disorder characterized primarily by dry mouth (xerostomia) and dry eyes (xerophthalmia). Hypofunction of the salivary and lacrimal glands causes discomfort and serious side effects, including increased susceptibility to infection. Over-the-counter saliva and tear substitutes provide only short-term relief and often fail to prevent long-term complications. Pilocarpine has been used successfully to treat the signs and symptoms of radiation-induced dry mouth, but not the dryness symptoms associated with Sjogren's syndrome. Vivino and associates evaluated the safety and effectiveness of pilocarpine tablets in treating dry mouth and eyes in patients with Sjogren's syndrome.

Patients eligible for the study had a primary or secondary diagnosis of Sjogren's syndrome, with significant dry mouth and eyes. Before entry into the study, patients were required to stop using any electric device to produce saliva and to discontinue using any medications that caused dry mouth. Patients with clinically significant cardiac, renal or gastrointestinal conditions, diabetes mellitus or multiple sclerosis were excluded from the study. Those who met the study criteria were randomized to receive either 2.5 mg or 5 mg of pilocarpine, or placebo tablets four times daily for 12 weeks. Efficacy was measured by response to questionnaires and measurements of salivary flow obtained at follow-up visits six and 12 weeks after initiation of the study. Safety was evaluated based on results of laboratory and diagnostic studies.

Of the 373 predominantly female patients enrolled in the study, 121 were in the 2.5-mg group, 127 were in the 5-mg group and 125 were in the placebo group. Demographic characteristics were similar across groups. Within six to 12 weeks after pilocarpine therapy was initiated, patients in the 5-mg group reported significant overall improvements in the symptoms of eye and mouth dryness compared with the other groups. These patients also slept better and reported improvements in dry skin and nasal dryness. Salivary flow also increased significantly in this group compared with the other groups. No serious drug-related adverse effects were reported, although some patients reported mild or moderate sweating, flushing and urinary frequency.

The authors conclude that treatment with the higher dosage of pilocarpine significantly improved the dryness associated with Sjogren's syndrome and that these benefits clearly outweighed the mild to moderate adverse cholinergic activity. Almost any patient with some degree of exocrine gland dysfunction in association with Sjogren's syndrome could potentially benefit from this treatment.

Barbara Apgar, M.D., M.S.

Vivino FB, et al. Pilocarpine tablets for the treatment of dry mouth and dry eye symptoms in patients with Sjogren syndrome. Arch Intern Med January 25, 1999:159:174-81.

COPYRIGHT 1999 American Academy of Family Physicians
COPYRIGHT 2000 Gale Group

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