SJOGREN’S SYNDROME THE DRY DISEASE

7 min


Sjogren’s syndrome is a chronic autoimmune disease. An autoimmune disorder occurs when the body’s immune system instead of killing the foreign bodies destroys the body’s healthy cells. A syndrome is a group of signs and symptoms that occur together and indicates a condition. Hence, this article highlights Sjogren’s syndrome which is an autoimmune disorder.

SJOGREN’S SYNDROME:

Sjogren’s syndrome is a chronic, systemic autoimmune disorder that affects the salivary glands and lacrimal glands. This results in dry mouth (xerostomia), dry eyes (xerophthalmia).

HISTORY OF SJOGRENS SYNDROME:

Henrik Samuel Conrad Sjogren was a Swedish ophthalmologist. He once examined a female patient who has dry eyes and a dry mouth. He described it as ‘a general disease’ of the nosologic entity. Further, he also described the histological and clinical components of the syndrome. And therefore, he termed this condition Sjogren’s syndrome.

OTHER NAMES OF SJOGREN’S SYNDROME:

  • Sicca syndrome
  • Keratoconjunctivitis Sicca
  • Gougerot Sjogren’s syndrome
  • Dacryosialoadenopathia atrophicans
  • Secreto-inhibitor- xerodermostenosis.

PATHOGENESIS OF SJOGRENS’S SYNDROME:

The cause of Sjogren’s syndrome is unknown. Though it is not a hereditary disease, there is a genetic influence. In addition, certain histocompatibility antigens (HLA’s) found in greater frequency in these patients. Anti Ro(SS-A) and Anti La(SS-B) antibodies are present in the serum of these patients. This is a classical feature of this disease. Researchers have suggested that viruses such as Epstein Barr virus (EBV), human T cell lymphotropic virus, cytomegalovirus (CMV), paramyxovirus may cause this disease. But the evidence for this is still speculative.

EPIDEMIOLOGY:

Sjogren’s syndrome is the third most common rheumatic autoimmune disorder. The condition has been reported worldwide. More common in women. Almost 3.1 million adults have Sjogren’s syndrome. It affects 0.5-1.0% of the total population

CLINICAL FEATURES OF SJOGREN’S SYNDROME:

AGE GROUP:

This disease predominately occurs in women over 40 years of age. Although children or young adults may be affected. The female to male ratio is approximately 10:1.

CLINICAL FEATURES:

The typical clinical features of the disease are:

  • Dryness of mouth (xerostomia)
  • Dryness of eyes (xerophthalmia)
  • The presence of xerostomia and xerophthalmia alone is termed Sicca syndrome.
  • The dryness occurs as a result of the hypofunction of the salivary glands and lacrimal glands.
  • This results in a burning sensation of the oral mucosa.
  • Since the oral cavity is completely dry without saliva, extensive dental caries is more common in these patients.
  • The nasal mucosa becomes dry.
  • Parotid gland enlargement ( that is swelling below the ear lobe region).
  • Lymphadenopathy is more common.
  • Dryness affects various secretory glands of the nose, larynx, pharynx, and tracheobronchial area.

TYPES OF SJOGRENS SYNDROME:

 Traditionally there are two types of sjogren’s :

  • Primary Sjogren’s
  • Secondary Sjogren’s

PRIMARY SJOGRENS:

  • Characterized by Sicca syndrome alone ( that is dry eyes and dry mouth alone).
  • No other autoimmune disorder is present.
  • Lymphadenopathy is more common in primary Sjogren’s.
  • Therefore, 80% of the patients get affected by this type.

SECONDARY SJOGRENS:

In this type, there is Sicca syndrome associated with autoimmune diseases like rheumatoid arthritis.

Rheumatoid arthritis is an integral part of this type (14% cases)

Patients affected with Sjogren’s and rheumatoid arthritis have a different clinical presentations like,

  • Parotid enlargement (that is swelling below the ear lobe region)
  • Lymphadenopathy (that is a condition affecting lymph nodes, resulting in enlargement of lymph nodes.)
  • Raynaud phenomenon (a condition in which blood flow to the fingers, toes gets interrupted resulting in numbness of hands and toes) 
  • Affects the kidneys

REVISED INTERNATIONAL CRITERIA FOR SJOGRENS SYNDROME:

OCULAR SYMPTOMS:

  • If the patients have any one of the below symptoms like:
  • Dry eyes for more than 3months
  • The recurrent sensation of sand or gravel in the eyes.
  • If the patient uses artificial tear substitutes more than three times a day.

ORAL SYMPTOMS:

  • If the patient has a dry mouth for more than 3 months.
  • Presence of enlarged or swollen salivary glands.
  • Frequent consumption of liquid foods aids in swallowing dry foods.

AUTO ANTIBODIES:

  • Presence of autoantibodies in the serum such as
  • Anti Ro (SS-A) or La (SS-B)antibodies, or both in the serum.

SALIVARY GLAND INVOLVEMENT:

  • Unstimulated salivary gland flow (less than 1.5 ml in 15 minutes)
  • Presence of salivary calculi in parotid sialgogrephy.

If the above criteria satisfy the condition, then the patient will be diagnosed with Sjogren’s syndrome. And also there are separate criteria for primary and secondary Sjogren’s. That is also based on the above-revised criteria.

DIAGNOSTIC TEST FOR SJOGREN’S SYNDROME:

SCHIRMER’S TEST:

This diagnostic test used to confirm the decreased tear secretion. A standardized sterile filter paper placed in the lower eyelid. Hence, this can be assessed by measuring the wetness of tear production in the filter paper. Therefore, a value below 5mm (after 5 mins) considered abnormal.

ROSE BENGAL TEST:

The rapid staining test used to stain the ocular surface of the eyes. To find the epithelial damage. Rose Bengal is a purple stain, the cornea portion of the eyes (the white part), should not take up the dye. If it turns purple it indicates abnormality.

LABORATORY INVESTIGATIONS FOR SJOGREN’S SYNDROME:

ERYTHROCYTE SEDIMENTATION TEST(ESR):

This type of blood test measures the erythrocytes (red blood cells) in the blood sample. And also, this test depends on how quickly the erythrocytes settle in the bottom of the test tube. Therefore, The ESR value is usually high in Sjogren’s patients.

SERUM IMMUNOGLOBULIN TEST:

Immunoglobulins are also known as antibodies. This is a quantitative immunoglobulin test that determines the abnormal levels of IgG, IgA, and IgM in the blood. The IgG level is usually high indicates infection. If the level of IgG, IgM, IgA are too low also indicates infection. Similarly, the level of IgG is usually high in this condition. Hence, this immunoglobulin test is one of the best methods to determine autoimmune diseases like rheumatoid arthritis, etc.

AUTO ANTIBODIES TEST(ANA):

This test determines the severity of the autoimmune disease. This test determines specific antibodies in the bloodstream, there is the presence of Anti Ro and Anti La antibodies in the blood sample of these patients. These are the specific antibodies for this condition. Hence this confirms Sjogren’s syndrome.

BIOPSY:

A biopsy is the removal of tissue from the affected area for the purpose of microscopic examination and diagnosis. This biopsy taken in the lower lip region and labial salivary gland biopsy is widely indicated for this condition. Further, lymphoepithelial differentiation is one of the classic histopathological features of this condition.

MANAGEMENT OF SJOGREN’S SYNDROME:

  • Kerato conjunctivitis( dry eyes) is treated by the installation of ocular lubricants like artificial tears containing methylcellulose etc.
  • Pilocarpine increases salivary secretions in the oral cavity.
  • Xerostomia (dry mouth) is treated by salivary substitutes that increase salivary flow in the oral cavity. Artificial salivary substitutes contain xylitol, hydroxyethylcellulose, etc.
  • And also, products like artificial salivary chewing gums and candies increase the salivary flow.
  • Due to xerostomia, extensive dental caries is more common in these patients. So scrupulous oral hygiene and frequent fluoride application are indicated.
  • For dry nasal mucosa use nasal drops
  • In case of discomfort, surgery is needed.

TIPS FOR SJOGRENS PATIENTS:

  • Foods like caffeinated drinks, alcohol will keep your mouth dry, avoid these drinks.
  • Foods that contain more acidic content, citrus fruits, spicy foods should be avoided as they can irritate the dry mouth resulting in a sore mouth. Avoid these foods.
  • Avoid sugary food. Consuming high sugary foods also increases caries.
  • Modify the texture of your foods. This will help you to swallow easily.
  • Try to sip a drink while eating, consume more porridge kinds of foods. This will help you to swallow easily.
  • Consume an anti-inflammatory diet. This diet includes omega 3 fatty acids rich foods like fishes, fatty fishes, avocadoes, olive oil. All types of dark-coloured fruits and vegetables extremely rich in vitamins and minerals. Therefore, fibre-rich foods increase bowel movements.
  • Add more garlic and turmeric to your diet. Both have extremely beneficial effects.
  • Further, avoid foods that trigger the inflammatory response. For example, red meat, highly saturated foods, refined carbohydrates, preservatives like artificial sweeteners.

CELEBRITIES AFFECTED WITH SJOGREN’S SYNDROME:

The famous tennis player Venus Williams was diagnosed with Sjogren’s syndrome in 2011. For few days she stopped her practice. After treatment and adopting a vegan diet, she is absolutely fine now. On the other hand, not only treatment, but diet also plays a major role in this condition.

Megan Park was also diagnosed with Rheumatoid arthritis.

CONCLUSION:

To sum up, this is the third most common type of autoimmune disorder in the world. The majority of the cases have been reported with rheumatoid arthritis. By following the treatment and adopting to anti-inflammatory diet plan we can overcome this syndrome. Therefore avoid caffeinated drinks, high sugary foods, acidic foods, citrus fruits in your diet. This might worsen the condition.


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Dr Shalini S.G
I am a dental surgeon. I love travelling and listening to music and a passionate person in research and doing lots of presentation. My area of interest is pathology, diagnosis and forensics.