Sjogren’s Syndrome -Features, Diagnosis, Self-Care Tips, and Treatment

Last Updated on September 1, 2020

Sjogren’s syndrome is an autoimmune disease characterized by two common symptoms – dry eyes and a dry mouth.

In autoimmune diseases, the immune system of the body mistakenly identifies certain parts of one’s own body as foreign invaders. In response, it releases special antibodies, called autoantibodies that attack a person’s own cells and tissues. These autoantibodies can attack and damage the connective tissue and a variety of other organs (skin, joints, muscles, etc).

In Sjögren syndrome, the immune system mainly attacks the glands that produce tears (the lacrimal glands) and saliva (the salivary glands), thus impairing the ability of these glands to secrete these fluids.

Sjogren’s syndrome has been reported in all areas of the world. It is the second most common rheumatic autoimmune disorder, after rheumatoid arthritis and systemic lupus erythematosus (SLE).

Various studies indicate that the disease has a prevalence of 3% of the population.

This disease can affect people of any age, but it most commonly affects middle-aged people between the ages of 40 and 60. Women are affected much more commonly than men.

Having a first-degree relative with an autoimmune disease increases the risk of having Sjogren’s syndrome. The inheritance pattern of this predisposition is however unknown.

Read more about Genetic Inheritance: Modes and Significance

No differences in disease prevalence due to race and ethnicity are known.

Almost half of the patients also have symptoms of other autoimmune diseases like rheumatoid arthritis, lupus, etc.

Types of Sjogren’s Syndrome

Primary Sjogren’s syndrome (pSS) which occurs by itself.

Secondary Sjogren’s syndrome (sSS) occurs as a part of other autoimmune diseases. sSS can occur along with rheumatoid arthritis, systemic lupus erythematosus, progressive systemic sclerosis, and less frequently with multiple sclerosis, autoimmune hepatitis, and thyroiditis. Arriving at a correct diagnosis is often difficult in patients with sSS because the symptoms of various disease entities overlap.

Clinical Features

The two characteristic features of Sjogren’s syndrome include:

• Dry eyes (Keratoconjunctivitis sicca): The eyes may have a burning, itchy, or gritty feeling as if there is sand in the eyes. Patients may have constant discomfort of the eye, blurred vision, light sensitivity, or even corneal damage.
• Dry mouth (Xerostomia): The mouth feels dry, making it difficult to speak, swallow, eat, or digest food. There is an increased chance of developing infections of the mouth, tooth infections, and tooth decay.

Besides other organs of the body may be affected.

• Swelling, pain or stiffness of the joints
• Swollen salivary glands — especially located beneath the jaw and in front of the ears
• Dry skin and skin rashes
• Dry throat or lips causing a change in taste, hoarseness of voice and recurrent infections of the mouth
• Dryness of nose causing a change in smell
• Persistent dry cough or shortness of breath
• Vaginal dryness
• Prolonged fatigue
• Trouble concentrating or remembering things
• Headache
• Heartburn or acid reflux

Complications of Sjogren’s Syndrome

The majority of the patients with Sjögren’s syndrome remain healthy, but some people may develop complications.

The most common complications of Sjogren’s syndrome involve your eyes and mouth.

Eye complications: If dry eyes are not treated, it may lead to corneal damage or even permanent loss of vision.

Dental complications: Dental caries and chronic oral infections

Organ dysfunction: Sjogren’s syndrome may lead to inflammation of the various body organs

• Lungs may be involved causing pneumonia, bronchitis, etc.
• Kidney involvement may result in autoimmune tubulointerstitial nephritis leading to urinary concentrating defect, and distal renal tubular acidosis.
• Liver may be affected resulting in hepatitis or cirrhosis
• Inflammation of the blood vessels or vasculitis can result in a rash that appears like small bruises or reddish-purple spots
• Peripheral neuropathy resulting in numbness, tingling or burning of hands and feet

Pregnancy complications: Babies born to women with Sjogren’s syndrome having anti-Ro/SS-A and anti-La/SS-B antibodies are at an increased risk of complications. There is a greater risk of developing neonatal lupus erythematosus which is a type of rash that occurs in newborns and lasts for a few weeks. Also, the baby is more prone to develop congenital heart diseases like heart block.

Cancer. A small percentage of people (5%) may develop non-Hodgkin lymphoma – NHL (cancer derived from white blood cells). The most common NHL are salivary extranodal marginal zone B cell lymphomas (MALT lymphomas) and diffuse large B-cell lymphoma.

Thus, it is important for all patients to be under regular medical supervision and follow-up.

Diagnosis

The disease is diagnosed based on clinical signs and symptoms, complete physical examination, blood tests, and special investigations.

Dry eyes and dry mouth which are early and important signs of the disease may be caused by many other conditions such as aging or due to certain medications. Special tests may be helpful to assess any reduction in tear or saliva production. Blood tests are used to detect the presence of autoantibodies which are a characteristic feature of autoimmune diseases.

Blood tests

Complete blood count

A raised total leukocyte count is indicative of infections.

ESR

Levels may be raised in chronic infections.

Liver function tests and kidney function tests

Values may be abnormal if these organs are involved.

Autoantibodies

These include

• Antinuclear Antibodies (ANA)]
  • Typical SS ANA patterns are SSA/Ro and SSB/La.
  • May be seen in other autoimmune diseases as well
  • 70% of Sjögren’s patients are positive for SS-A while 40% are positive for SS-B.
  • Anti-SSB/La is much more specific for Sjogren’s syndrome.
• Rheumatoid factor (RF)
  • Sjogren’s syndrome occurs frequently with rheumatoid arthritis
  • RF antibodies may be detected in these patients.

Eye tests

Slit-lamp examination

The test carried out by an ophthalmologist can detect reduced tear film in the eyes.

The Rose Bengal test

This test is used to measure the functioning of the lacrimal glands. A non-toxic dye – Rose Bengal is instilled in the eyes.

In case of dry eyes or a complete lack of tear film, the dye is taken up by the healthy epithelial cells and the eyes will display extensive staining with Rose Bengal.

Schirmer’s test

It is a test used to measure the degree of dryness of the eyes. A small piece of filter paper is kept inside the lower eyelid for five minutes to measure the production of tears. The wetness is then measured with a ruler.

If the moistened area is less than 5 mm, it usually indicates Sjogren’s syndrome. Other eye-related conditions and medications being used are also taken into consideration for analyzing the results.

Unstimulated whole saliva flow collection

The person is asked to spit as much saliva as possible into a cup every minute for 15 minutes and the amount of saliva collected is then weighed.

This test helps to determine whether the salivary glands are working normally or not. Less than 1.5 ml of saliva collected in 15 minutes is considered a positive result and could indicate Sjogren’s syndrome.

Imaging tests

Contrast sialography

A contrast dye is injected into the salivary duct which helps to visualize the salivary gland and gives an idea about how much saliva flows into the mouth.

Salivary scintigraphy

A radioactive isotope is injected into a vein which is then tracked. The uptake and excretion of this radioactive dye by the salivary gland are evaluated which indicates the functioning of the salivary gland.

Salivary gland biopsy

The biopsy of the minor salivary gland obtained from the patient’s lip was once considered the gold standard for the diagnosis of Sjogren syndrome. However, it is not necessary for diagnosis in all cases. It is useful in cases where the diagnosis is in doubt.

A small amount of tissue is removed from the inner lip and examined under a microscope. The presence of lymphocytes clustered around salivary glands and damage to these glands from the inflammatory cells is suggestive of Sjogren syndrome.

Read more about Types of Biopsies and Their Applications

Read more about Light Microscope: Parts, Usage, Handling. and Care

Self-care Tips

• Avoid dry or windy places as they can aggravate dryness of eyes. You can also use a humidifier near you to keep the surrounding air moist.
• Wear wraparound sunglasses or glasses with sides so as to prevent drying of eyes.
• Reduce your screen time, watching TV, or spending a long time reading as it tends to make the eyes dry.
• Avoid medicines that can cause dryness of eyes as a side effect.
• The use of humidifiers and nasal saline irrigation is helpful in case of nasal dryness.
• Practice good oral hygiene.
• Brush your teeth twice a day.
• Use anti-bacterial mouthwash.
• Chew sugar-free chewing gum, suck on ice cubes or use saliva substitutes to help relieve dry mouth.
• Avoid sugary foods or drinks.
• Drink water frequently.
• Stop smoking.
• Avoid alcohol.
• Avoid medicines that can cause dry mouth.
• Have a dental check-up at least once in 6 months.

Treatment

There is no definite cure for Sjogren’s syndrome. The main aim of treatment is to relieve the symptoms of dry eyes and dry mouth and also to prevent and treat long-term complications mainly infections.

Eyecare

• Artificial tears act as moisture replacement therapy and help to relieve symptoms of dry eyes. The drug, cyclosporine may be prescribed in cases of chronic dry eye as it suppresses the inflammation that disrupts tear secretion.
• In severe cases, a minor surgical procedure that seals the tear ducts that drain tears from the eyes (punctal occlusion) may be carried out to relieve dry eyes.
• Punctal plugs (made of collagen or silicon) may be inserted to retain tears on the surface of the eye.

Dental care

• Drugs such as pilocarpine (Salagen) and cevimeline (Evoxac) help to stimulate and increase the production of saliva.
• Yeast infections in the mouth are treated with antifungal drugs.

Dry skin may be treated by using a moisturizing cream (emollient) once or twice a day.

Vaginal dryness may be treated with lubricants which are liquids or gels put inside the vagina. Hormonal treatment such as estrogen may also be considered.

Drugs that reduce gastric acid production (eg. H2 blockers or proton-pump inhibitors) are used to treat symptoms of heartburn or acid reflux.

Swelling and pain in the joints are treated with NSAIDs or other arthritis drugs.

Hydroxychloroquine (Plaquenil), a drug used to treat malaria may be helpful in some patients with Sjogren’s syndrome as it reduces joint pain and skin rash which occurs in some patients.

Serious systemic symptoms such as liver, lung, or kidney problems will require specific treatment. Treatment with corticosteroids such as prednisone and/or immunosuppressive agents like methotrexate, azathioprine, or cyclophosphamide may be required in certain cases.

The role of rituximab (Rituxan) and other biological therapies in patients of Sjogren’s syndrome where the entire body is affected is being evaluated.

Prognosis

About 5% of patients of Sjogren syndrome may develop non-Hodgkin lymphoma – NHL (cancer derived from white blood cells). More severe cases are much more likely to develop lymphomas than mild or moderate cases.

Besides this, patients of Sjogren’s syndrome do not usually experience significant organ related dysfunction and the mortality rates are only slightly increased in comparison with the general population.

References