What Is Sjogren’s Syndrome?
Sjogren's syndrome is a condition consisting of a triad of conditions, like keratoconjunctivitis sicca, xerostomia, and rheumatoid arthritis. Subsequently, it has been found that some patients present only with dry eyes and dry mouth, while others also developed systemic lupus erythematosus, polyarteritis nodosa, polymyositis, or scleroderma and rheumatoid arthritis.
Sjogren's syndrome was clinically illustrated as an autoimmune disorder of exocrine glands, and it is of two types:
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Primary Sjogren's syndrome is associated with salivary and lacrimal dysfunction without any autoimmune condition.
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Secondary Sjogren's syndrome is characterized by:
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Lacrimal gland involvement causing ocular dryness.
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Salivary gland involvement causing decreased salivation (xerostomia).
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Along with autoimmune disorders such as rheumatoid arthritis and systemic lupus erythematosus.
What Are the Causes of Sjogren’s Syndrome?
As it is an autoimmune disorder, the immune system attacks the body's own cells mistakenly. The clear cause is still unknown, but specific genes put people at higher risk of the disorder. Still, a triggering mechanism like an infection with a particular virus or bacteria is also necessary. Various causes of this disease have been suggested, such as,
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Genetic.
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Hormonal.
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Infectious.
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Immunological.
The immune system will first target the glands that produce tears and saliva. Still, it can damage other parts of the body (thyroid, joints, kidneys, lungs, liver, skin, and nerves). It may be possible that a combination of extrinsic and intrinsic factors plays a role in the etiology of this condition. An altered immunological response is the main intrinsic factor responsible for the disease. Cytomegalovirus, paramyxovirus, and Epstein-Barr virus have been implicated in the pathogenesis of this condition but have not been proven conclusively.
What Are the Clinical Manifestations of Sjogren’s Syndrome?
This disease occurs predominantly in postmenopausal women over 40 years of age, although children or young adults may also be affected. The female to male ratio is approximately 10:1. The typical features of the disease are dryness of the mouth and eyes as a result of the hypofunction of salivary and lacrimal glands. This often results in painful, burning sensations of the oral mucosa. In addition, various secretory glands of the nose, larynx, pharynx, and vagina are involved in this dryness. Dryness of the pharynx, larynx and nasal cavity may lead to pneumonia.
Lacrimal gland involvement gives rise to dryness of the eyes and continuous feeling of dirt or foreign body in the eye. Conjunctivitis and corneal ulceration may be indicative of Sjogren's syndrome. Xerostomia (severe dryness of the oral cavity) causes difficulty in speech, mastication, and deglutition. Saliva will appear thick and ropy, and during clinical examination by the dentist, the mirror head will get stuck to the tongue and mucosa of the oral cavity. The lips will appear dry and cracked with angular cheilitis, and the tongue appears depapillated and at times lobulated. Candidiasis and increased dental caries are most commonly observed.
Parotid gland enlargement, lymphadenopathy, purpura, Raynaud’s phenomenon, kidney involvement, and myositis are more frequently manifested in people without rheumatoid arthritis.
How Is Sjogren’s Syndrome Diagnosed?
Sjogren's syndrome can be challenging to diagnose because the symptoms vary from one person to another, and the symptoms can be similar to other diseases. The adverse effects of some medications also mimic symptoms of Sjogren's syndrome. So, it is diagnosed by the following tests that can help to rule out other conditions from Sjogren's syndrome.
1. Blood tests:
The doctor takes blood tests to check levels of different blood cells, antibodies, inflammatory conditions, and problems with the liver and kidneys.
2. Ophthalmic Tests:
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Schirmer's Test - In normal patients, 15 mm of the filter paper gets wet when placed in the lower conjunctival sac for five minutes, but in the case of Sjogren's syndrome, only 5 mm of the filter paper gets wet.
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Rose Bengal Dye Test - Denuded and damaged areas of the cornea can be visualized clearly with this dye.
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Break Up Time Test - A slit-lamp is used, and an interval between complete blink and appearance of a dry spot on the cornea is noted.
3. Salivary Gland Tests:
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Sialography - It shows thinning of the ducts and a decrease in the number of ductules. It appears as punctate, globular, and cavitary pseudo sialectasis. It is termed pseudo sialectasis because the appearance is due to the pooling of the dye in the periductal area and not because of the dilation of the ductules. The overall description is given by the term branchless tree with a fruit-laden appearance (Rankow).
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Salivary Scintigraphy - It is the nuclear medicine test, and it involves the injection of a radioactive isotope into a vein, which is tracked for an hour to see when it arrives at the salivary gland.
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MRI (Magnetic Resonance Imaging) - It shows the salt and pepper appearances of the enlarged salivary glands.
4. Biopsy
A lip biopsy is done to detect if there are any clusters of inflammatory cells, which is indicative of Sjogren's syndrome. A sliver of tissue will be removed from salivary glands in the lip and examined under the microscope.
What Are the Treatment Options for Sjogren’s Syndrome?
There is no satisfactory treatment for Sjogren's syndrome. Most patients are treated symptomatically and medical consultation for the prevention of pneumonia is needed.
1. To Manage Keratoconjunctivitis and Xerostomia - A regular examination by the ophthalmologist is needed. Keratoconjunctivitis is treated by a mixture of ocular lubricants such as artificial tears containing Methylcellulose, and xerostomia is treated by saliva substitutes with topical Methylcellulose (Oralube, Xero-Lube wet mouth) or such as those used in the treatment of a person with xerostomia secondary to radiation therapy.
2. Dental Considerations - Extensive dental caries are a common complication, so oral hygiene and frequent fluoride application are indicated to reduce this problem. Topical application of Nystatin and Clotrimazole is needed to control candidiasis. There is no specific treatment for the enlargement of salivary glands.
3. Surgery - Surgery has been employed but is generally recommended only in patients with discomfort. A minor procedure to close the tear ducts that drain tears from the eyes may help relieve the dry eyes. Silicone plugs or collagen are inserted into the ducts to help preserve the tears. Also, patients with Sjogren's syndrome may be on steroids or immunosuppressants for the treatment of systemic lupus erythematosus or rheumatoid arthritis; therefore, one must take precaution against infection preceding oral surgical procedures.
