Sjogren’s Syndrome

What is Sjogren’s Syndrome?

Sjogren’s Syndrome is an autoimmune disease  where the body’s natural defences attack the normal cells and tissues present in one’s body. The syndrome brings about inflammation of tissues and glands in the body. There is a higher prevalence of Sjogren’s syndrome in females than males. Statistics suggest that 90% of Sjogren’s cases are females. The syndrome is generally deemed to be complicated when infections of the eyes, airway passages and mouth persist.  

Sjogren’s syndrome is generally portrayed by the characteristic dryness of mouth and eyes which arise due to the syndrome. An autoimmune disease is characterised by the manufacturing or production of supplementary antibodies (in blood) which in turn attacks or fights against normal tissues in the body. Thus, one can say the immune system of a person is “misdirected or reversed” as the sole purpose of the immune system is to fight against foreign particles and substances. This misdirected immune system gives rise to inflammation of tissues. The autoimmune disease, Sjogren’s syndrome, results in the inflammation of glands that produce saliva and tears. Hence, an inflammation of the lacrimal glands that produce tears leads to a reduction in the normal water production for tears leading to dry eyes. Dry lips and mouth are due to the inflammation of salivary glands that functions to produce saliva.  Inflammation can also take place in the parotid glands.

There are different conditions which correlate to different Sjogren’s syndrome. Primary Sjogren’s syndrome is the inflammation of glands that do not involve or associate with other connective tissue diseases. Secondary Sjogren’s syndrome, on the other hand, involve connective tissue disease namely systemic lupus erythematosus, rheumatoid arthritis, scleroderma, etc. Furthermore, sicca syndrome is the characteristic dryness of eyes and mouth that may be experienced by individuals who do or do not have Sjogren’s syndrome.

  • Causes of Sjogren’s Syndrome
  • Signs and Symptoms of Sjogren’s Syndrome
  • Diagnosis of Sjogren’s Syndrome
  • Treatment for Sjogren’s Syndrome
  • Preventative Measures 
  • Prognosis for Sjogren’s Syndrome
  • CONCLUSION

 

Causes of Sjogren’s Syndrome

The root cause which leads to one’s suffering from Sjogren’s syndrome is yet to be found. New research and studies are concentrating on finding genetic factors which may lead to the syndrome. There is considerable consideration over the causes of the Sjogren’s and scientists are actively participating in this area of research to find a genetic cause as some family members of the patient are sometimes found to be affected by the syndrome as well. Background research on family medical history has also shown that sufferers of Sjogren’s syndrome have family members who suffer from other autoimmune diseases like systemic lupus erythematosus, thyroid disease, diabetes (Type I), etc  arising due to autoimmune conditions

Signs and Symptoms of Sjogren’s Syndrome

Symptoms that indicate Sjogren’s syndrome can be the inflammation of the glands as mentioned above. In addition there is a possibility the syndrome can also affect other organs of the body called extra-glandular manifestations.

Inflammation of the lacrimal gland which is involved in the production of tears due to Sjogren’s syndrome lead to dryness of eyes. But this is not it, the dryness can in turn bring out irritation of the eye, reduced production of tears from the gland, infection and can lead to injury of the cornea. Xerophthalmia is a medical term used to refer to ‘dry eye’ conditions. Dry eyes lead to infections too.

Furthermore, when the salivary glands are inflamed it results in dryness of the mouth, difficulty in swallowing food, cavities, dental caries, gum disease and  sores in the mouth. In addition, it can give rise to infection of the parotid glands present in the cheeks. As a consequence of the inflammation lip dryness occurs too, along with dryness of the mouth. Xerostomia is a medical term used to refer to ‘dry mouth’ conditions.

Moreover, inflammation can occur in other glands of the body which is quite rare in Sjogren’s syndrome.  Inflammation can occur along the airway passages which concurrently leads to lung infection. Inflammation can also occur in the vagina which once again lead to vaginal infections and pain during intercourse.

Extra glandular is a term which means “outside of the glands”. Extra glandular problems can occur and generally include fatigue, joint pain, arthritis due to inflammation, Raynaud’s phenomenon, inflammation of the lungs, enlargement of lymph nodes as well as muscle, nerve and kidney disorders  Another rare extra glandular problem related to Sjogren’s syndrome is blood vessel inflammation referred to as vasculitis. This will result in tissue damages that are supplied by these inflamed vessels.

Other common diseases that are usually linked to Sjogren’s syndrome are thyroiditis and gastroesophageal reflux disease. Thyroiditis, is an autoimmune disease, known as Hashimoto’s thyroiditis, leads to abnormal thyroid hormone levels in the body which are detected by appropriate blood tests. Gastroesophageal reflux or GERD , on the other hand, brings about heart burn and swallowing difficulties. Moreover, primary biliary cirrhosis is an autoimmune disease which is rare and serious and are not commonly linked to Sjogren’s syndrome. It is a disease of the liver which results in liver tissue scarring. Sjogren’s syndrome can also lead to cancer of the lymph glands known as lymphoma. The likelihood of this happening is quite small and may happen after several years of suffering from Sjogren’s. Thus, it is important for a patient to report any unusual  swelling of the lymph nodes to a medical doctor.

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Diagnosis of Sjogren’s Syndrome

Diagnosis of Sjogren’s syndrome generally involves looking out for certain clinical features such as dry eyes and dry mouth. Dryness of the eyes can be easily be diagnosed by a doctor by the simple use of a Schirmer’s test using Schirmer tear test strips. This test assess the ability of the eye to produce tears, just enough to keep the eyes moist. The test is performed by placing a small Schirmer testing strips under the eye lids and then evaluating the ability of the eye to wet this piece of paper. Other professional and sophisticated tests can be undertaken by an eye specialist known as an ophthalmologist.

Sjogren’s syndrome that involves the salivary glands can make these glands bigger, tough or soft. To find out if there is significant inflammation of the salivary glands, you can use radiologic nuclear medicine salivary scans. In addition, the “slacken” production of saliva by the inflamed salivary glands can be found out by salivary flow testing. Then again, a strong diagnosis can be made with the help of a biopsy of the tissue in the salivary gland.

From time to time, glands of the lower lip are taken to acquire a biopsy of the salivary gland tissue which are used to diagnose Sjogren’s syndrome. This procedure requires the patient to be under local anaesthesia. The lower lip salivary gland biopsy sample is then obtained by a surgeon who will make a small incision or slit on the lower lip. The surgeon will then remove a small amount of sample tissue from within.

Sjogren’s syndrome is an autoimmune disease thus the patient will produce extra antibodies which will fight against one’s own normal body tissue. These antibodies are called autoantibodies and can be usually detected through a simple blood test that will utilise the properties of antinuclear antibodies or ANA. ANA are present in all patients. SS-A and SS-B antibodies (Sjogren’s syndrome A and B antibodies) are common antibodies that can be generally found in most patients diagnosed with Sjogren’s but not all. Other common antibodies are rheumatoid factor and thyroid antibodies. SS-A and SS-B antibodies are sometimes called Ro and La antibodies. Furthermore, low red blood count or anaemia and abnormal level of inflammation markers like sedimentation rate and C-reactive protein can also be found in a patient diagnosed with the syndrome.

 Treatment for Sjogren’s Syndrome

Patients who have been diagnosed with Sjogren’s syndrome can be treated by directing treatment towards the parts of the body that has been affected as well as trying to prevent occurrences of complications such as infection. Unfortunately, no cure has been found that can effectively treat the syndrome.

As mentioned before, dryness of eyes is a characteristic of Sjogren’s syndrome. This can be managed by the use of artificial tears, application of eye lubricants which can be used overnight and also by reducing or completely stopping the use of hair dryers which can contribute to eye dryness. When eye dryness become a more serious complication, a visit to the ophthalmologist becomes necessary who would plug the tear duct in order to make tears last longer. Cyclosporine eyedrops or Restasis are medical eye drops that are approved by doctors which functions to minimise inflammation of lacrimal or tear glands. Thereby, the optimal function of the glands are achieved. Conjunctivitis which is a type of eye infection may arise. Some  signs of conjunctivitis that may arise include increased eye redness, pain and secretions such as puss. When these signs manifest a visit to a medical doctor becomes a must. Flaxseed oil inclusion in one’s diet is also proven to be beneficial in dryness of the eyes.

Dryness of the mouth is also another complication related to Sjogren’s syndrome which can be managed by consuming abundant fluids, using a humidifier to humidify the air and avoiding tooth decay and cavities by practising good dental care and regularly visiting the dentist. Salivary glands can be stimulated to produce saliva. This can be achieved by asking the patient to suck on sugarless lemon drops or glycerine swabs. Medications too can be prescribed to stimulate saliva production. Pilocarpine (Salagen) and cevimeline (Evoxac) are medications used for salivary gland stimulation. However, these medications can only be prescribed to some individuals, not all. People who suffer from certain heart diseases, asthma or glaucoma should not be prescribed the above mentioned medications. Another technique that can be applied for this condition is the preparation of artificial saliva that can relieve the complications that arise due to dry mouth. These types of agents can be easily be obtained. They are readily available as “over-the-counter products” (toothpaste, gum and mouthwash). Numoisyn Liquid and lozenges can be utilised as part of the treatment plan to treat the condition. Vitamin E oil has also shown to be helpful but only to some extent. Severe complications can be avoided by avoiding infections and treating infections of the mouth and teeth as early as possible. Thus, a proper dental care plan is crucial. In the case of the inflammation of the parotid gland, moist warm compresses can be used to manage pain and swelling.

Dryness of the airway passages can be managed by using nasal sprays in the form of saline (saltwater). If the patient suffers from vaginal dryness then a vaginal lubricant should be used when necessary.

Hydroxychloroquine (Plaquenil) has also been administered to Sjogren’s syndrome sufferers. It helps in fatigue, muscle and joint pains. Other severe complications of Sjogren’s syndrome like vasculitis can be managed by immune-suppression drugs. Prednisone (Cortisone) and/or azathioprine (Imuran) or cyclophosphamide (Cytoxan) are all examples of immune-suppression drugs.

On the other hand, infections which can be a severe complication of Sjogren’s syndrome can be handled with relevant antibiotics. Lymphoma which is the cancer of the lymph nodes is a rare condition that can arise and is often and usually treated independently.

 Preventative Measures 

As Sjogren’s syndrome is thought to be linked to genetics and thus inherited; currently there is no mechanism to prevent the condition from arising. Having mentioned that, preventative measures are available for the complications that arise due to the syndrome like infection and eye irritation. Management and prevention of these complications were outlined in the previous section.

Prognosis for Sjogren’s Syndrome

The prognosis for Sjogren’s syndrome is quite excellent when proper care and attention is taken to manage the intricacies related to the syndrome.  Dryness of the eyes have a tendency to lead to serious eye injuries. Thus, it is crucial to take care of one’s eyes by visiting an eye specialist. At the same time, decaying of teeth and infections of the parotid glands can arise due to mouth dryness. Therefore, excellent dental care and hygiene is required to keep these complications at bay. However, other severe complications like biliary cirrhosis and lymphoma can have a major impact on the prognosis. These complications require regular visits to medical care facilities. 

 

 

CONCLUSION

The name Sjogren comes from a Swedish ophthalmologist, Henrik Sjogren. He, in the early 1900s, called it the “keratoconjunctivitis sicca”. Sicca syndrome is now only used to refer to any complication that arise due to the dryness of mouth and eyes. Therefore, sicca is a term used to describe the dryness of mouth and eyes.

Ongoing active research is currently being done for Sjogren’s syndrome. There is hope that many other effective medications and treatments will be available to the patient in the near future. Research, which was done recently, has shown that rituximab (Rituxan) is effective in treating many manifestations that arise due to Sjogren’s syndrome.

 

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