Sjogren’s Syndrome

Symptoms Of 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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Types Of Sjogren's Syndrome

Sjogren’s syndrome is classified into two forms, namely:

1. Primary form: In this type, the syndrome is not associated with any type of rheumatic disease. This form develops as a result of any other health condition.

2. Secondary form: This form is associated with other rheumatic diseases, such as rheumatoid arthritis, systemic lupus erythematosus, scleroderma (hardening and tightening of the skin), polymyositis (muscle weakness affecting both sides of the body), or psoriatic arthritis (arthritis associated with psoriasis - red patches of skin topped with silvery scales).

Causes Of Sjogren's Syndrome

Sjogren’s syndrome is mostly thought to be an autoimmune disease, in which the immune system attacks the healthy cells instead of the diseased ones as a result of some triggers. In this syndrome, the immune system attacks the ophthalmic glands in the eyes and salivary glands in the mouth.

Though the exact cause of the syndrome is still unclear, Sjogren's syndrome is thought to be a result of a combination of genetic and environmental factors. According to several researchers, many genes affect the risk of developing Sjogren's syndrome, but that development of the condition may be triggered by something in the environment as well.

In particular, viral or bacterial infections can activate the immune system, and cause the development of Sjogren's syndrome in susceptible individuals.

Risk Factors For Sjogren's Syndrome

Though the explicit cause of this syndrome is still not clear, several kinds of research suggest the abnormal functioning of the immune system causing damage to the healthy cells and tissues. There can be several factors playing a prominent role in it like the environmental and genetic factors. An individual developing Sjogren's syndrome has most likely inherited the risk from one or both of their parents, along with certain environmental impacts like a viral or bacterial infection.

Some of the common traits most commonly in people that act as risk are:

1. Age: Generally seen in individuals over the age of 40, but it can affect younger individuals, including children.

2. Gender: Several types of research have been done to understand the gender predilection of Sjogren's syndrome. Women are 10 times more likely to develop this disease than men and it may be due to the effect of sex hormones on a woman’s immune system.

The role of the hormone estrogen that drops after menopause is said to be the major risk factor in the development of this disease in women. 

3. Rheumatic disease: The risk of developing Sjogren's syndrome is more in individuals who already have a rheumatic condition 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.

4. Genes: Scientists are beginning to identify the genes that can be related to Sjogren's syndrome by analyzing large samples of DNA from the patients suffering from the disease. There are several different genes that have been identified which can increase the risk of developing Sjogren's syndrome.

Diagnosis Of Sjogren’s 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:

1. Schirmer test

It is done to estimate the functioning of the lacrimal glands to measure the production of tears. A wetting paper strip of ≤ 5 mm is placed under the lower lid for 5 mins, after which the paper is removed and checked for its moisture content. 

2. Cornea and conjunctiva examination

This test is done to check for any punctuate or filamentary keratitis lesions, which is a typical feature of keratoconjunctivitis sicca. It is done using a rose bengal or lissamine green stain.

3. Salivary analysis

It is done to estimate the production of saliva from an unstimulated salivary gland. The value is considered normal if there is > 1.5 ml in 15 minutes.

4. Biopsy of minor salivary gland

This is done to assess the presence of lymphocytic infiltrates around salivary gland epithelium which is a hallmark of Sjogren's syndrome.

5. 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 done include:

• Complete blood count (CBC): It is a group of tests done to get information regarding the blood cells like red blood cells (RBC), white blood cells (WBCs) and platelets.

• Chemistry panel: They help evaluate the body's electrolyte balance and the status of several major body organs.

• Chest radiography: Chest x-ray uses a very small dose of ionizing radiation to produce pictures of the inside of the chest.

• Protein electrophoresis: Protein electrophoresis is a test to measure specific proteins in the blood. 

• Antinuclear antibodies test: This test looks for antinuclear antibodies in the blood to detect any autoimmune disorder. 

• Antibodies against Ro/SSA and La/SSB autoantigens: This test is done to detect autoantibodies directed against Ro/SSA and La/SSB autoantigens generally indicative of patients with Sjogren's syndrome. 

• Rheumatoid factor: Rheumatoid factors are proteins and high levels of it in the blood are most 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) is a virus that attacks the body's immune system leading to acquired immunodeficiency syndrome (AIDS).

  • Human T-lymphotropic virus 1 was the first oncogenic human retrovirus to be discovered causing adult T-cell leukaemia/lymphoma and HTLV-1-associated myelopathy or tropical spastic paraparesis (a progressive nervous system condition).

• Antibod against thyroid antigens:These antibodies play a key role in the diagnosis of autoimmune disorders.

• Thyroid function: Thyroid function is a group of tests that are done together to detect or diagnose thyroid disease and consist of thyroid stimulating hormone (TSH), thyroxine (T4), and triiodothyronine (T3). 

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

Prevention Of Sjogren’s Syndrome

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 the doctor to understand, if the patient is suffering from any other rheumatic condition like:

• Lupus

• Rheumatoid arthritis

• Scleroderma

• Polymyositis

• Psoriatic arthritis

Specialist To Visit

Sjogren’s syndrome comes with a varied amount of symptoms. These doctors diagnose and manage oral symptoms like dryness of the mouth, dental caries, periodontal disorders, eye diseases and any other rheumatic diseases that are related to the syndrome. They are:

• Dentist

• General physician

• Ophthalmologist

• Rheumatologist

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Treatment Of Sjogren’s Syndrome

Once the diagnosis is done, the doctors will develop a comprehensive treatment plan that will depend on the extent and severity of the symptoms. Most symptoms can be treated with over-the-counter products whereas some may require prescription medications or minor surgical procedures.

While some individuals may only experience dry eyes and dry mouth, there will be systemic symptoms such as difficulty in concentrating, memory lapses, fatigue, and joint pain. In some cases, the disease can also affect the blood vessels and internal organs like the lungs and the 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 consists of the insertion of tiny plugs into the tear duct by the ophthalmologist to block them. It is done to sustain tears in the eyes longer, which will help keep them moist.

Dry mouth and its related symptoms 

There are a lot of over-the-counter options that are available to relieve the symptoms of dry mouth. They are: 

• Sugarless candy 

• Sugar-free gum 

• Artificial saliva products such as a spray or lozenge

If this does not help, the patient may consult a dentist or a general physician to get prescribed medications like: 

• Pilocarpine: Pilocarpine stimulates certain nerves and glands in the body thereby increasing the production of saliva, tears, sweat, digestive juices, and other secretions.

• Cevimeline: 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

Home-care For Sjogren’s Syndrome

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

Tips to protect eyes and relieve the symptoms

Dryness of the eyes is a very common symptom of Sjogren’s syndrome. The patient affected with it often feels a burning sensation or like there is 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 their glasses

• Avoid windy, dry, or smoky places

• Reduce reading, watching TV, or looking at screens for a long time

Tips for dryness of the mouth

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

• Chew sugar-free gum

• Try sucking on candies 

• Keep sipping water during the day

• Use artificial saliva products such as a spray or lozenge

• Quit smoking

• Avoid caffeine

• Limit alcohol use

• Decrease the sugar intake

• Take over-the-counter saliva substitutes or oral lubricants 

• Maintain good oral hygiene by:

  • Always 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 a dental cleaning.

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

• If the patient wears oral dentures, it has to be made sure dentures are disinfected every night.

Tips for other symptoms

Sjogren’s syndrome can have an effect on the whole body. Some individuals have vaginal dryness, dryness of the lips, fatigue, rashes on the skin, or joint pain. Sjogren’s syndrome can also cause inflammation of other organs like the kidneys or lungs. Some of the measures that can help in aiding against these symptoms are:

• For dry lips: Use petroleum jelly and lip balms every 2 hours.

• For vaginal dryness: This is 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 the pain.

• For dry skin: This can be managed easily by using body lotions regularly and throughout the day, especially after baths or showers.

• For blocked nasal passages or 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 happens due to a lack of saliva that helps reduce the acidity of the stomach. It can be managed with dietary modifications and non-prescription medications.

Complications Of Sjogren’s Syndrome

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 the other parts of the body like lungs, liver, kidney, lymph nodes, or nerves are:

• Pneumonia 

• Bronchitis

• Kidney disorders

• Hepatitis 

• Liver cirrhosis

• Lymphoma

• Peripheral neuropathy (numbness, tingling, and burning in your hands and feet)

• Inflammation in lung, kidney and liver

• Vasculitis (an inflammation of the blood vessels)

• Lymph node swelling

• Interstitial lung disease

• Cystic lung disease

• MALT lymphoma (belongs to a group of non-Hodgkin lymphomas called marginal zone lymphomas)

• Loss of pregnancy

• Neonatal lupus syndrome

• Parotid tumors

Alternative Therapies For Sjogren’s Syndrome

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

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 given either as a sole treatment or in conjunction with conventional therapy to improve lacrimal and salivary gland function. This option is considerably safe and further research has been going on 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. There are a few things that can be added and avoided to manage the symptoms. They are:

• 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 the food

• Use a drinking straw to make swallowing easier

• Add cucumber to diet to increase fluid intake

The best option is to follow a balanced diet which contains fresh fruit and vegetables and has levels of fats and sugar. Some foods like artificial sweeteners are believed to trigger an inflammatory reaction so they must be avoided.

Living With Sjogren’s Syndrome

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

• Monitor any development of severe complications, like lymphoma, vasculitis, and pulmonary, kidney, and liver disease

• Lookout for any kind of pain or redness in the eyes and get it evaluated promptly, as this may signal an infection or corneal damage

• Follow proper protocols for dry mouth such as regular use of fluoride and maintenance of oral hygiene

• Get dental care at regular intervals to reduce the risk of cavities and other dental problems

• Visit the physician regularly for general health screening and to know about preventative medicine

• Pay close attention to any abnormal or persistent swelling in the glands around the face or neck, under the arms, or in the groin areas, as that might be a signal for lymphoma

• Use moisturizing products to tackle the dryness of the skin and vagina

• Give importance to diet and exercise for general well-being