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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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Lymphoma of the parotid gland in Sjogren's syndrome
From Ear, Nose & Throat Journal, 3/1/04 by Enrique Palacios

Sjogren's syndrome is a chronic autoimmune disorder that manifests as a lymphocytic infiltration of the exocrine glands, particularly the salivary and lacrimal glands. The hallmark of Sjogren's syndrome is the sicca complex of xerostomia and keratoconjunctivitis. (1) Its association with lymphoma is well documented; this severe complication of primary Sjogren's syndrome occurs in 5 to 10% of patients who are followed for more than 10 years. (2, 3) Lymphomas that develop in patients with Sjogren's syndrome are classified as mucosa-associated lymphoid tissue (MALT) lymphomas. MALT lymphomas are sub-classified as either low-grade or high-grade. (4)

We evaluated a 60-year-old woman who had experienced an insidious and slowly progressive development of dry eyes and dry mouth. She exhibited no evidence of underlying rheumatoid arthritis. Clinically, a soft-tissue mass was identified in the parotid area. Computed tomography (CT) at the level of the parotid glands showed a nodular appearance of both parotid glands, indicating dilation of the acini (figure, A). Also noted was a low-density soft-tissue mass in the right parotid gland that measured approximately 3 cm at its greater dimension (figure, B); this lesion was histologically proven to be a lymphoma.

[FIGURE OMITTED]

The pathophysiology of lymphoma in Sjogren's syndrome remains unknown. To date, there is no argument favoring a viral infection or deregulation of a unique oncogene or antioncogene. (2) CT and magnetic resonance imaging can demonstrate findings consistent with Sjogren's syndrome. In such cases, the parotid glands have a punctate or nodular appearance that represents globular collections. These findings, however, are nonspecific; they are also seen in chronic sialadenitis and granulomatous diseases. (4)

References

(1.) Talal N. Sjogren's syndrome In: Schumacher HR, Jr., ed. Primer on the Rheumatic Diseases. 9th ed. Atlanta: Arthritis Foundation, 1988:136-8.

(2.) Mariette X. [Gougerot-Sjogren syndrome. Risk of lymphoma]. Presse Med 1999;28:1214-18.

(3.) Voulgarelis M, Moutsopoulos HM. Lymphoproliferation in autoimmunity and Siogren's syndrome. Curr Rheumatol Rep 2003;5:317-23.

(4.) Som PM, Brandwein MS. Salivary glands: Anatomy and pathology. In: Som PM, Curtin HD, eds. Head and Neck Imaging. 4th ed., vol. 2, St. Louis: Mosby, 2003:2005-133.

>From the Department of Radiology, Louisiana State University Health Sciences Center, New Orleans.

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