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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.

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The Saxon Test In Diffuse Lung Disease - Sjogren's syndrome diagnosis - Abstract
From CHEST, 10/1/00 by Toshikazu Kurumagawa

Toshikazu Kurumagawa, MD(*); Hideo Kobayashi, MD,FCCP and Kazuo Motoyoshi, MD. Third Department of Internal Medicine, National Defense Medical College, Tokorozawa, Saitama, Japan.

PURPOSE: Sjogren's syndrome is a chronic inflammatory disease characterized pathologically by lymphocytic infiltration to the exocrine glands. It has also been found to affect the respiratory system in many forms. Therefore, patients with diffuse lung disease might have Sjogren's syndrome, even if no clinical sicca syndrome. We explored the potential involvement of Sjogren's syndrome as associated pathogenesis of diffuse lung disease.

METHODS: A prospective clinical study was performed with measurement of saliva production using the Saxon test. Patients, who were suspected diffuse lung disease and did not exhibit obvious xerosis, were examined from 7/97 to 3/00. Patients treated parasympathomimetic blocking or psychotropic agents, or had already been diagnosed Sjogren's syndrome were excluded from this study. Patients with decreased saliva production, which was determined by the value under 2 g/min, in the Saxon test, followed by the Schirmer test, fluorescent test, salivary gland scintigram, sialography, labial biopsy, and serologic tests (Ro/SSA, La/ SSB). Medical records were also reviewed.

RESULTS: The eligible cases were 149 (male: 64, female: 85) and Mean [+ or -] SD age was 55.3 [+ or -] 14.8 year-old (from 25 to 80). The group included 64 patients with sarcoidosis, 30 with various interstitial pneumonias, 15 with bronchial lesion, 10 with non-tuberculosis mycobacterial infection, 8 with COPD, 4 with primary pulmonary lymphoma, 3 with chronic eosinophilic pneumonia, and 15 with miscellaneous diseases. Decreased saliva production was detected in 29 (19.5%), 17 out of 29 cases had accomplished subsequent further examinations after the Saxon test, then Sjogren's syndrome was diagnosed in 8 (5.4%), and suspected in 4(2.7%). Cases diagnosed Sjogren's syndrome revealed 2 sarcoidosis, 2 interstitial pneumonia, primary pulmonary lymphoma, bronchiolitis, pulmonary hypertension, and undetermined interstitial lesion.

CONCLUSION: Prevalence of Sjogren's syndrome was higher among patients with diffuse lung disease in this study than the historical studies (0.05-3.3%). Sjogren's syndrome was accompanied by diffuse lung disease, frequently.

CLINICAL IMPLICATIONS: In patients with diffuse lung diseases, it is always important to differentiate Sjogren's syndrome, even if the sicca syndrome was not apparent. In our study, the Saxon test proved an effective screening procedure for this purpose.

COPYRIGHT 2000 American College of Chest Physicians
COPYRIGHT 2001 Gale Group

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