Sjogren’s syndrome is an immune-mediated disease primarily affecting exocrine glands. The glands mostly affected are Salivary glands and lacrimal glands.
Sjogren’s syndrome may be classified as
1. Primary Sjogren’s Syndrome
2. Secondary Sjogren’s Syndrome
Primary disease is idiopathic with no extra exocrine manifestation. However the secondary Sjogren’s disease is associated with other exocrine tissue disorders.
According to Sjogren’s Society of Canada, as many as 430,000 citizens of Canada mostly women live with Sjorgen’s Syndrome
What are the Sign and Symptoms of Sjogren’s syndrome?
The pathology is always exocrine, involving lacrimal and Salivary glands
a. Ocular Involvement:
The lacrimal gland involvement leads to ocular symptoms such as itching and burning sensations of the conjunctiva and eye lids, redness and foreign body sensation and increased sensitivity to light. The most frequent symptom is the dryness of eyes called Xerophthalmia
Upon examination, there is enlargement of the lacrimal gland and dilatation of the vessels of cornea
b. Mouth Involvement
There is deceased production of salivary fluid leading to dry mouth called Xerostomia. Other infrequent symptoms might be sore throat, adhering of food to buccal mucosa and sometimes dysphagia.
The sings in oropharyngeal involvement include disappreance of saliva pool from the mouth floor, and the disappearance of papillae from the surface of tongue. The lips appear to be cracked, peeling and atrophic.
c. Other Exocrine Gland Involvement:
Sicca syndrome is referred to Sjogren’s syndrome when only exocrine glands are involved. As stated earlier, the most frequent glands involved are lacrimal and salivary, however sicca syndrome may involve vagina leading to vaginal dryness and tracheal dryness leading to chronic bronchitis
d. Connective tissue involvement:
Secondary Sjogren’s syndrome is associated with involvement of body’s connective tissue. The frequently affected organs apart from eyes and mouth are muscles, liver, pancreas, skin, lungs and brain leading to various signs and symptoms
e. Association with other medical conditions:
Sjogren’s syndrome is mostly associated with other autoimmune conditions such as celiac disease, autoimmune thyroiditis, multiple sclerosis and fibromyalgia.
What Causes Sjogren’s syndrome?
Many risk factors are identified to contribute to cause Sjogren syndrome. Few important are
- Genetical Factors
- Hormonal Factors
- Microchimerism factors
- Environmental Factors
What is the Pathogenesis of Disease?
Sjogren’s disease is autoimmune in origin. An important study published in the Journal of Nature Reviews Rheumatology shows that the immune complexes deposit and destroy parenchyma of the glands leading to decreased secretion. This leads to signs and symptoms of the disease such as Xerostomia and Xerophthalmia.
How is the disease Diagnosed?
In 2004, Sjogren’s syndrome foundation issued guidelines to cut the time of diagnosis into half. Various diagnostic tests are described here
a. Specific Tests for Oropharyngeal Involvement:
Minor Salivary gland biopsy is highly sensitive and specific to diagnose the disease. There is peri-ductal lymphocyte infiltrate in the biopsy specimen.
Sialography is done to measure to flow of the saliva in the ducts.
b. Specific tests for ocular involvement:
Schirmer test is done to evaluate Xerophthalmia. A filter paper is placed in the inferior fornix of eye. Wetting of less than 5mm in 5 min makes the test positive
Rose Bengal test is done to evaluate corneal damage. A rose Bengal solution in applied in the inferior fornix of the eye. The eyes are blinked twice followed by slit lamp examination. In positive case, destroyed conjunctival epithelium and retention of rose Bengal stain is seen under slit lamp.
c. Other non-Specific Tests:
CBC shows normocytic normochromic anemia while leukopenia
ESR, CPK, Gamma Globulin and Liver enzymes might be raised.
There might be proteinuria with renal tubular acidosis.
In most of the cases, anti-nuclear antibody, RA factor and Anti Ro and anti-LA antibodies are positive.
The complement levels are mostly decreased
How the disease can be treated?
There is no specific cure or treatment of the Sjogren’s syndrome. The disease is treated symptomatically. The current approach to treatment according to a clinical study is described below
- Moisture replacement therapy is done such as artificial tear and punctal plugs. The punctal plugs retain most of the tear in the eyes. Similarly, stimulation drugs can be used to produce more tears such as pilocarpine. Immunosupprsents such as Azathioprine are given to retard the progression of gland destruction
- In women, painful sexual intercourse is resulted from vaginal dryness. Local Lubricants are used during sexual intercourse
- Steroids are systemic immune suppressants are used to retard the progression of disease. They have been effective sometimes, however no clinical trial has established its definitive role to cure and treat the disease.
Prognosis of the Disease
Most of the cases remit spontaneously, however study shows that the mortality rate is due to the progression of the disease in 5% of the case into Non-Hodgkin Lymphoma and another MALT lymphoma.
Sjogren’s syndrome is an autoimmune disorder primarily involving eyes and mouth. The disease is diagnosed clinically mostly. There is no specific treatment however steroids and immunosuppressants are sometimes proven beneficial.