What is Sjögren's Syndrome?
Sjögren's syndrome is a chronic autoimmune disorder in which the body's immune system attacks its own moisture-producing glands. It majorly affects the salivary glands (mouth) and the tear glands (eyes), causing them to dry up.
This disease can also affect other organs, and it is pronounced as “SHOW-gren’s syndrome”.
Types of Sjögren's Syndrome
There are two types of Sjögren's syndrome:
- Primary: It develops on its own without the presence of any underlying autoimmune disease.
- Secondary: It develops due to another autoimmune disease, such as Rheumatoid arthritis, Systemic sclerosis, and Systemic lupus erythematosus
Symptoms
The main symptoms of Sjögren's syndrome are:
- Dry mouth: A feeling of the throat stuffed with cotton, making swallowing or speaking difficult. Some people also have a chalky feeling in the mouth.
- Dry eyes: A burning or itching sensation in the eyes with grittiness.
Other symptoms associated with the disease are:
- Dryness of skin, nose, and lips
- Vaginal dryness
- Persistent dry cough
- Inflammation of the face and neck glands
- Swelling and joint pain
- Brain fog
- Muscle pain or weakness
- Fatigue
- Tooth decay
- Loss of taste
- Burning sensation from the stomach to the chest
Causes
Different causes of Sjögren's syndrome are:
- Genetics: Some people may have a gene that causes their immune system, especially the white blood cells, to attack the healthy cells instead of the harmful ones.
- Virus or Bacterial Infection: The infection caused by the virus and bacteria activates the white blood cells to fight against it, but sometimes they get confused and start attacking the healthy cells located in the salivary and tear glands.
Risk Factors
Anyone can develop Sjögren's syndrome, but a certain group of people is more prone to develop it:
- Sex: Women are more likely to develop the disease than men
- Age: Develops in people between the ages of 45 and 55
- Other Autoimmune Diseases: People who have at least one other autoimmune disease can develop it
- Family History: A family history increases the likelihood of developing the disease
Complications
Various complications can be caused by Sjögren's syndrome:
- Oral and eye infections
- Lymphomas (cancerous tumors in the lymph nodes)
- Lung problems
- Oral and eye infections
- Brain, spine, or nerve problems
Many people with Sjögren's syndrome live their lives without any severe complications. If you have secondary Sjögren's syndrome, you are at risk of developing complications. It is suggested to talk to your healthcare provider to understand the management.
How is Sjögren's Syndrome Diagnosed?
It is difficult to diagnose Sjögren's syndrome because the symptoms vary from person to person and can be confused with many other related or unrelated conditions. Side effects of some medicines also mimic the signs and symptoms of this disease.
Some diagnostic tests to identify the condition are:
Blood Tests
- To check the abnormal count of red and white blood cells
- To check the presence of inflammatory conditions
- To check the abnormal functioning of the liver and kidneys
- To check the presence of antibodies
Eye Tests
- A Schirmer tear test is done to measure the number of tears produced
- Slit-lamp test is done to check cornea damage caused by dryness in the eyes
Lip Biopsy
- A lip biopsy is done to detect the presence of clusters of inflammatory cells
- A small amount of tissue is removed from the lip salivary gland and examined under the microscope
Imaging
- An imaging test called Sialogram is performed, which involves injecting a dye into the salivary glands in front of the ears. It shows the amount of saliva that flows into the mouth.
- A Salivary scintigraphy test is performed in which a radioactive isotope is injected into the vein, which is tracked for an hour to see how quickly it arrives in all salivary glands.
Management and Treatment
Sjögren's syndrome symptoms can be relieved by taking proper care, like using over-the-counter eyedrops and sipping water more frequently. But if you are still facing difficulty in your daily routine, your healthcare provider will recommend some treatment options:
Medications
As per the symptoms, your doctor might suggest some medicines that:
- Decrease eye inflammation: Eyedrops such as cyclosporine (Restasis) or lifitegrast (Xiidra)
- Increase saliva production: Drugs such as pilocarpine (Salagen) and cevimeline (Evoxac)
- Manage arthritis symptoms: Nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications
- Suppress the overactive immune system: Methotrexate, Hydroxychloroquine, and other disease-modifying antirheumatic drugs (DMARDs)
Surgery
A minor surgery known as Punctal occlusion can be performed to seal the tear ducts from your eyes. Your healthcare provider will insert collagen or silicone plugs into the tear ducts to preserve your tears and might help relieve your dry eyes.
Prevention
It might not be possible to prevent Sjögren's syndrome, as it is an autoimmune disease, but symptoms can be managed by taking self-care measures. Below are some:
- Apply eye lubricants overnight to restore eye moisture
- Wear glasses when outside to protect your eyes from the direct blow of air and heat
- Quit smoking
- Drink water frequently to keep your mouth and lips hydrated
- Boost your saliva flow by consuming sugarless or citrus-flavored candies
- Use artificial saliva available in the form of sprays or lozenges to keep your mouth moist
- Keep your nose unclogged by using a nasal saline spray to avoid mouth breathing
- Maintain good oral hygiene to avoid cavities
Prevent drying up your skin by:
- Not using hot water for a shower
- Pat dry with a towel instead of rubbing
- Apply moisturizer
- Use vaginal lubricants for vaginal dryness
Takeaway
Sjögren's syndrome is a complex autoimmune disease leading to dry eyes and dry mouth. The exact cause is still unknown, and there is no proper cure for the disease. However, early diagnosis and a multidisciplinary approach can help manage the symptoms.
Long-term outcomes vary from person to person, but thanks to ongoing medical research and increasing awareness, people with Sjögren's syndrome are steadily improving. Regular monitoring and personalized care are vital to improve the quality of life and manage the disease progression.
