Sjogren’s Syndrome

Sjogren’s syndrome can affect the whole body and can cause inflammation of organs like the kidneys or lungs. The symptoms of Sjogren's syndrome include:

• Dryness of the eyes, mouth, and skin
• Dryness of the vagina
• Tiredness/fatigue
• Pain in the muscles and joints
• Swelling of the salivary glands
• Rashes on the skin, especially after sun exposure
• Dry nasal passages and throat along with a dry cough
• Acid reflux
• Swelling of the glands around the face and neck
• Trouble sleeping
• Poor concentration and memory problems
• Numbness, tingling, and weakness, especially in the extremities
• Shortness of breath or trouble breathing
• Muscle weakness

Note: The reason for dryness of the mouth (also called xerostomia) can be due to several other causes and not just Sjogren’s syndrome.

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Sjogren’s syndrome is primarily considered an autoimmune disease, where the immune system mistakenly attacks healthy cells instead of diseased ones due to certain triggers. In this condition, the immune system targets the ophthalmic glands in the eyes and the salivary glands in the mouth.

Although the exact cause of Sjogren's syndrome remains unclear, it is believed to arise from a combination of genetic and environmental factors. Several researchers indicate that multiple genes influence the risk of developing Sjogren's syndrome, while environmental factors may also play a role in triggering the condition.

Particularly, viral or bacterial infections can activate the immune system, potentially leading to the onset of Sjogren's syndrome in individuals who are genetically predisposed.

Though the explicit cause of Sjogren's syndrome is still not clear, several types of research suggest that abnormal functioning of the immune system causes damage to healthy cells and tissues. Several factors may play a prominent role, including environmental and genetic influences. An individual developing Sjogren's syndrome has most likely inherited the risk from one or both parents, along with certain environmental impacts such as a viral or bacterial infection.

Common risk factors include:

• Age: Generally seen in individuals over the age of 40, but it can also affect younger individuals, including children.
• Gender: Research indicates that women are 10 times more likely to develop Sjogren's syndrome than men. This may be due to the effect of sex hormones on the female immune system. The role of the hormone estrogen, which drops after menopause, is considered a major risk factor for the development of this disease in women.
• Rheumatic disease: The risk of developing Sjogren's syndrome is higher in individuals with existing rheumatic conditions such as lupus, rheumatoid arthritis, scleroderma, polymyositis, or psoriatic arthritis. About half of all individuals with Sjogren's syndrome have been diagnosed with another autoimmune disorder.
• Genes: Scientists are beginning to identify genes related to Sjogren's syndrome by analyzing large samples of DNA from affected patients. Several different genes have been identified that may increase the risk of developing this syndrome.

The major manifestation of Sjogren's syndrome is oral and ocular (eye) dryness. In clinical practice, patients who present with these symptoms should be assessed for it with the following tests:

• Schirmer test: This test estimates the functioning of the lacrimal glands to measure tear production. A wetting paper strip of ≤ 5 mm is placed under the lower lid for 5 minutes, after which the paper is removed and checked for moisture content.
• Cornea and conjunctiva examination: This test checks for any punctate or filamentary keratitis lesions, which are typical features of keratoconjunctivitis sicca. It is performed using a rose bengal or lissamine green stain.
• Salivary analysis: This test estimates the production of saliva from an unstimulated salivary gland. A value of > 1.5 ml in 15 minutes is considered normal.
• Biopsy of minor salivary gland: This procedure assesses the presence of lymphocytic infiltrates around the salivary gland epithelium, which is a hallmark of Sjogren's syndrome.
• Past medical history: If the patient has any pre-existing autoimmune disease, along with dry eyes and dry mouth, it can be concluded that the patient has developed secondary Sjogren’s syndrome.

Along with these, other investigations that may be performed include:

• Complete blood count (CBC): A group of tests that provide information regarding blood cells, including red blood cells (RBC), white blood cells (WBCs), and platelets.
• Chemistry panel: These tests help evaluate the body's electrolyte balance and the status of several major body organs.
• Chest radiography: A chest x-ray uses a very small dose of ionizing radiation to produce images of the inside of the chest.
• Protein electrophoresis: This test measures specific proteins in the blood.
• Antinuclear antibodies test: This test detects antinuclear antibodies in the blood to identify any autoimmune disorder.
• Antibodies against Ro/SSA and La/SSB autoantigens: This test detects autoantibodies directed against Ro/SSA and La/SSB autoantigens, which are generally indicative of patients with Sjogren's syndrome.
• Rheumatoid factor: Rheumatoid factors are proteins, and high levels in the blood are often associated with autoimmune diseases such as rheumatoid arthritis and Sjogren's syndrome.
• Viral testing for:
  • Hepatitis C virus: Can cause liver inflammation, sometimes leading to serious liver damage.
  • Human immunodeficiency virus (HIV): A virus that attacks the body's immune system, leading to acquired immunodeficiency syndrome (AIDS).
  • Human T-lymphotropic virus 1: The first oncogenic human retrovirus discovered, causing adult T-cell leukaemia/lymphoma and HTLV-1-associated myelopathy or tropical spastic paraparesis (a progressive nervous system condition).
• Antibodies against thyroid antigens: These antibodies play a key role in diagnosing autoimmune disorders.
• Thyroid function: A group of tests performed together to detect or diagnose thyroid disease, consisting of thyroid stimulating hormone (TSH), thyroxine (T4), and triiodothyronine (T3).

Once the diagnosis is established, additional investigational tests such as cryoglobulins, complement levels, and immunofixation can be performed in patients with peripheral purpura, peripheral neuropathy, salivary gland enlargement, or in situ demonstration of salivary gland lymphoma. Additionally, upper endoscopies, bone marrow biopsy, and computed tomography scans of the neck, thorax, and abdomen should be conducted to detect any potential development of lymphoma.

Since the actual cause for any autoimmune condition is still unknown, it is difficult to prevent it. Although Sjogren’s syndrome can be prevented to some extent by monitoring the risk factors and consulting a doctor to understand if the patient is suffering from any other rheumatic condition such as:

• Lupus
• Rheumatoid arthritis
• Scleroderma
• Polymyositis
• Psoriatic arthritis

Once the diagnosis is made, doctors will develop a comprehensive treatment plan based on the extent and severity of the symptoms. Most symptoms can be treated with over-the-counter products, while some may require prescription medications or minor surgical procedures. While some individuals may only experience dry eyes and dry mouth, systemic symptoms such as difficulty concentrating, memory lapses, fatigue, and joint pain may also occur. In some cases, the disease can affect blood vessels and internal organs like the lungs and kidneys. The treatment for various symptoms is discussed below:

Dry Eyes and Eye Inflammation

Dry eyes are the most common symptoms of this syndrome. Mild to moderate cases are managed by the following:

• Use of artificial tears during the day and a gel at night.
• Hot compresses and eyelid cleansers along with gentle eyelid massage to relieve blocked oil glands in the eyelids, in case of blepharitis (eyelid inflammation).
• Eye drops prescribed by an ophthalmologist.

In certain severe cases of dry eyes, the doctor might suggest a minor surgical procedure called punctal occlusion. This procedure involves the insertion of tiny plugs into the tear duct by the ophthalmologist to block them, helping to sustain tears in the eyes longer and keep them moist.

Dry Mouth and Its Related Symptoms

There are several over-the-counter options available to relieve the symptoms of dry mouth:

• Sugarless candy
• Sugar-free gum
• Artificial saliva products such as a spray or lozenge

If these do not help, the patient may consult a dentist or a general physician to obtain prescribed medications such as:

• Pilocarpine: Stimulates certain nerves and glands in the body, increasing the production of saliva, tears, sweat, digestive juices, and other secretions.
• Cevimeline: Also helps to increase the secretions of the exocrine glands.

Other Symptoms

Individuals with Sjogren’s syndrome often experience joint pain due to rheumatic disorders. They may require medications such as:

• Ibuprofen (nonsteroidal anti-inflammatory drugs or NSAIDs)
• Prednisone (low-dose steroids)
• Hydroxychloroquine (disease-modifying anti-rheumatic drug)
• Immunosuppressants
• Antifungals

Sjogren’s syndrome may lead to a variety of symptoms that can affect general well-being, but most of these symptoms are manageable with simple home care measures or lifestyle changes.

Tips to Protect Eyes and Relieve Symptoms

Dryness of the eyes is a very common symptom of Sjogren’s syndrome. Patients often experience a burning sensation or the feeling of something in the eyes. Here are a few options to help reduce tear evaporation by protecting the eyes from wind, air, and airborne particles:

• Wear wraparound sunglasses or goggles
• Add shields to the sides of glasses
• Avoid windy, dry, or smoky places
• Reduce reading, watching TV, or looking at screens for extended periods

Tips for Dryness of the Mouth

Dry mouth is another common symptom of this syndrome, which can increase the risk of cavities and make it more difficult to speak or swallow. Some tips to help relieve the symptoms include:

• Chew sugar-free gum
• Try sucking on candies
• Keep sipping water throughout the day
• Use artificial saliva products such as sprays or lozenges
• Quit smoking
• Avoid caffeine
• Limit alcohol use
• Decrease sugar intake
• Take over-the-counter saliva substitutes or oral lubricants

Maintain good oral hygiene by:

• Brushing and flossing after every meal or snack
• Consulting a dentist at least twice a year
• Using fluoride toothpaste, a fluoride rinse, or getting a professional fluoride treatment after dental cleanings

Individuals with Sjogren’s syndrome are at a higher risk of contracting fungal infections of the mouth, which can be treated with antifungal medications or medicated troches that dissolve slowly in the mouth.

If the patient wears oral dentures, it is essential to ensure they are disinfected every night.

Tips for Other Symptoms

Sjogren’s syndrome can affect the whole body. Some individuals may experience vaginal dryness, dryness of the lips, fatigue, skin rashes, or joint pain. It can also cause inflammation of other organs like the kidneys or lungs. Some measures that can help alleviate these symptoms include:

• For dry lips: Use petroleum jelly and lip balms every 2 hours.
• For vaginal dryness: This is often seen in women after menopause. Products like vaginal moisturizers, estrogen cream, Vitamin E oil, hyaluronic acid suppositories, and vaginal lubricants can be helpful. Applying sufficient artificial lubrication to both partners during intercourse can help prevent pain.
• For dry skin: This can be managed easily by using body lotions regularly throughout the day, especially after baths or showers.
• For blocked nasal passages or a dry nose: Try saline nasal sprays and treat any other causes of congestion, such as allergies and sinus infections, before they worsen.
• For acid reflux: This generally occurs due to a lack of saliva that helps reduce stomach acidity. It can be managed with dietary modifications and non-prescription medications.

The most common complication of Sjogren's syndrome is the involvement of the eyes and mouth. Here are some of the common complications of the syndrome:

• Dental cavities
• Dry mouth
• Yeast infections
• Gum diseases
• Vision problems
• Corneal damage due to dry eyes
• Light sensitivity
• Blepharitis (inflammation of the eyelids)

Some of the less common complications that might affect other parts of the body include:

• Pneumonia
• Bronchitis
• Kidney disorders
• Hepatitis
• Liver cirrhosis
• Lymphoma
• Peripheral neuropathy (numbness, tingling, and burning in the hands and feet)
• Inflammation in the lung, kidney, and liver
• Vasculitis (an inflammation of the blood vessels)
• Lymph node swelling
• Interstitial lung disease
• Cystic lung disease
• MALT lymphoma (a group of non-Hodgkin lymphomas called marginal zone lymphomas)
• Loss of pregnancy
• Neonatal lupus syndrome
• Parotid tumors

The basic management of Sjogren’s syndrome involves lifestyle modification and the use of alternatives to relieve common symptoms such as dry eyes and dry mouth. Along with home remedies and self-care, there are several options in alternative medicine that can provide relief:

1. Chinese Herbal Medicine
   Studies have analyzed Chinese herbal medicine (CHM) for the treatment of primary Sjogren’s syndrome (PSS). These studies suggest that CHM can be administered either as a sole treatment or in conjunction with conventional therapy to improve lacrimal and salivary gland function. This option is considered safe, and further research is ongoing to determine the exact efficacy of this therapy.
2. Herbs and Supplements
   The fundamental aim of this therapy is to “calm” the immune system and prevent it from causing further damage. Some of the herbs and supplements that can help alleviate the symptoms of Sjogren’s syndrome include:
   • Cysteine
   • Evening primrose oil
   • Gamma-linolenic acid (GLA)
   • Omega-6 fatty acids
   • Sulphur
3. Diet
   There is no special diet for Sjogren’s syndrome unless the doctor suggests something specific. However, there are a few recommendations for managing symptoms:
   • Avoid alcohol, spicy, or acidic food items that may cause irritation in the mouth.
   • Use sugar-free lozenges (xylitol) to help keep the mouth moist.
   • Add sauces, olive oil, or dressings to lubricate food.
   • Consume water or any drink alongside meals.
   • Use a drinking straw to make swallowing easier.
   • Add cucumber to the diet to increase fluid intake.
   The best option is to follow a balanced diet that includes fresh fruits and vegetables while maintaining appropriate levels of fats and sugars. Some foods, such as artificial sweeteners, are believed to trigger an inflammatory reaction and should be avoided.

Proper management of Sjogren’s syndrome helps to alleviate symptoms and prevent complications. People with this syndrome can live full lives with proper self-care and the guidance of knowledgeable healthcare professionals. Here are a few strategies that can assist in managing the symptoms:

• Monitor for severe complications, such as lymphoma, vasculitis, and pulmonary, kidney, and liver disease.
• Be alert for any pain or redness in the eyes and seek prompt evaluation, as this may indicate an infection or corneal damage.
• Follow appropriate protocols for dry mouth, including regular use of fluoride and maintaining oral hygiene.
• Receive dental care at regular intervals to reduce the risk of cavities and other dental issues.
• Visit your physician regularly for general health screenings and information about preventative medicine.
• Pay close attention to any abnormal or persistent swelling in the glands around the face, neck, underarms, or groin, as this may signal lymphoma.
• Use moisturizing products to address dryness of the skin and vagina.
• Prioritize diet and exercise for overall well-being.