What you need to know about Sjogren’s syndrome

Sjogren’s is a progressive immune-system disorder. It is an autoimmune condition, meaning that the immune system wrongly attacks healthy tissues and cells.

It occurs when saliva glands, tear glands, and other exocrine tissues are invaded by white blood cells, leading to reduced development of tear and saliva. Dryness in the mouth, eyes, skin, nose, upper respiratory tract, and vagina may result.

Some autoimmune diseases, including rheumatoid arthritis, systemic erythematosus lupus, and primary biliary cholangitis, are associated with it.

Primary Sjogren’s develops on its own, although another condition, such as lupus, co-occurs with secondary Sjogren’s. The symptoms are equivalent, and both may be severe.

Sjogren’s is a serious condition, but early treatment may mean that complications are less likely to occur and it is less likely to cause tissue damage. If treated, the disorder may typically be well handled by a person.

Sjogren’s may occur at any age, but most diagnoses are made after 40 years of age. The patient is female in 90% of cases, but it also affects males and may be under-diagnosed.

In the United States, it affects about 0.1 to 4 percent of individuals.

Important facts about Sjogren’s

  • Sjogren’s is an autoimmune disorder that affects moisture-producing glands.
  • The condition affects 0.1 to 4 percent of people in the U.S., and 90 percent of people with Sjogren’s are women.
  • It causes the eyes and mouth to dry out, and can also lead to tooth decay, recurring oral thrush, and a persistent dry cough.
  • Sjogren’s affects multiple systems and can be difficult to diagnosis. However, blood tests, ophthalmological testing, and measuring the salivary flow rate can help identify the condition. A biopsy of the lower lip can be important in the diagnosis, especially if the hallmark autoantibodies SSA (Ro), and SSB (La) are negative, which can occur in up to 30% of biopsy positive cases.
  • Prescribed eye drops, artificial tears, and moisture chamber spectacles can help to relieve dry eyes.
  • Other medications can help to stimulate the flow of saliva. Chewing sugar-free gum can help lubricate the mouth, and numerous saliva substitutes are available to try.

Symptoms

Sjogren’s causes painful and irritating dryness of the eyes
Sjogren’s causes painful and irritating dryness of the eyes as well as other symptoms.

Failure to provide moisture for the eyes and dry mouth, accompanied by joint pain (arthritis/arthralgia), is the most common symptom associated with Sjogren’s.

Vaginal dryness can also be reported by females.

Other symptoms can include:

  • tooth decay, and eventual loss of teeth
  • persistent dry mouth
  • persistent dry cough
  • problems chewing and swallowing
  • hoarse voice
  • difficulty speaking
  • swollen salivary glands
  • recurring oral thrush, a fungal infection in the mouth

The signs and symptoms associated with dry eyes include:

  • a sensation in one or both eyes similar to the irritation caused by foreign material, such as sand or gravel
  • tired-looking and heavy eyes
  • an itchy sensation
  • constant feeling of irritation in the eyes
  • persistent dry eyes
  • discharge of mucus from the eyes
  • photophobia, or sensitivity to light
  • stinging or burning eyes
  • swollen and irritated eyelids
  • blurred vision

Symptoms may be aggravated by smoking, air travel, overhead fans, and air-conditioned or windy conditions.

In certain cases, other areas of the body are attacked by a patient’s immune system, causing the following signs and symptoms:

  • general tiredness
  • brain fog
  • aching muscles
  • inflammation of joints, as well as stiffness and pain
  • swelling in the salivary glands between the jaw and ears
  • peripheral neuropathy, or numbness and occasional pain in the arms or legs
  • small fiber neuropathy, which can be diagnosed by a skin biopsy
  • lung disease (bronchiolitis, interstitial lung disease, cystic lung disease)
  • Raynaud’s phenomenon, in which the hands feel painful, cold and numb
  • vasculitis, where the blood vessels become inflamed

Diet

Unless the doctor recommends one, there is no specific diet for Sjogren’s.

However, alcohol should be avoided. People may also wish to avoid foods, such as spicy or acidic products, that irritate the mouth.

Sugar-free (xylitol) lozenges can help keep the mouth moist.

To make food easier to swallow:

  • use sauces, olive oil, or dressings to lubricate food
  • have a drink alongside your food
  • use a drinking straw to make swallowing easier
  • put cucumber in a sandwich, to add moisture
  • increase fluid intake

Choosing a healthy diet high in fresh fruit and vegetables and low in saturated fats and sugar is the best choice. Some ingredients, such as artificial sweeteners, are suspected to cause an inflammatory reaction. It might be best to stop them.

It may be helpful to maintain a food diary to recognise any particular foods that may cause a reaction or exacerbate symptoms.

Treatment

Eye drops and artificial tears are effective methods of managing dry eyes.
Eye drops and artificial tears are effective methods of managing dry eyes.

The treatment of Sjogren is aimed at lubricating the infected areas and preventing the onset of complications.

Treatment options include:

  • Medications to stimulate saliva flow: These include pilocarpine and cevimeline. They have a short-term impact, limited to a few hours only, so several doses a day are usually required.
  • Artificial saliva: Saliva substitutes and mouth-coating gels can relieve dryness of the mouth. They are available as sprays, pre-treated swabs, and liquids. These can be important at night, as the mouth becomes dryer during sleep.
  • Artificial tears: Artificial tears can help to lubricate the eyes, and are available over-the-counter (OTC). Prescription options are available that reduce the need for artificial tears, such as cyclosporine ophthalmic emulsion and hydroxypropyl cellulose pellets.
  • Prescription eye drops: These include cyclosporine and lifitegrast.
  • Moisture chamber spectacles: These are special glasses that keep out irritants and retain moisture.
  • Prescription expectorants and throat medications: In cases of respiratory dryness, drugs such as those used to stimulate saliva flow, as well as linseed extract, sorbitol, xylitol, or malic acid, can moisturize the area.
  • Nonsteroidal anti-inflammatory drugs (NSAIDs): Anti-inflammatory drugs such as aspirin, naproxen, and ibuprofen can provide relief for people with Sjogren’s who are experiencing joint pain.
  • Disease-modifying antirheumatic drugs (DMARDs): In cases where the joint pain is accompanied by fatigue and rashes, DMARDs may relieve symptoms. Examples include hydroxychloroquine or methotrexate. If Sjogren’s seems to be affecting the muscles, nerves, lungs, or kidneys, stronger DMARDs, corticosteroids, or rituximab may be prescribed.
  • Antifungal medication: If oral yeast infections are occurring, medication may be prescribed to combat the fungus.
  • Vaginal lubricants: For vaginal dryness, water-based vaginal lubricants can provide a solution, especially during sexual intercourse.
  • Punctual occlusion: When all conservative treatment options have been exhausted, this surgical option seals the tear ducts with small plugs to reduce the drainage of tears from the eye. This keeps the eye moist for longer. Temporary silicone plugs are likely to be used until the procedure is confirmed successful.
  • Autologous eye serum: In severe cases of eye dryness, eye drops can be made from the person’s blood serum.

Here are some easy ways to keep the mouth lubricated.

  • consuming more liquids.
  • rinsing the mouth regularly to prevent infection and soothe the area
  • maintaining excellent oral and dental hygiene
  • topical fluoride gels or varnish
  • quitting smoking, as smoke irritates the mouth and speeds up the evaporation of saliva
  • chewing sugar-free gum, which stimulates the production of saliva
  • applying coconut oil to the dry areas, as it is both moisturizing and anti-microbial

Diagnosis

It can also be difficult to diagnose, since Sjogren’s signs and symptoms are similar to certain other health conditions. Different health practitioners may be seen by the patient, perhaps a dentist for dry mouth and tooth decay, a vaginal dryness gynecologist, and an ophthalmologist for dry eyes, a breathlessness and persistent cough pulmonologist.

Some drugs can also cause symptoms that are similar to Sjogren’s.

The conditions that will be used in a clinical trial for a definitive diagnosis include:

  • the need to apply eye drops containing tears more than 3 times a day
  • persistent dry eyes and mouth for over 3 months
  • evidence of dry eye in ophthalmological test results
  • measuring salivary flow rates
  • SSA or SSB autoantibodies in blood
  • a lip biopsy showing focal lymphocytic inflammation

Tests

Sjogren’s presents in different ways in different people. Some more tests may be ordered by a doctor, including:

Blood tests: Sjogren’s allows special antibodies to be found in the blood. Because only about 60 to 70 percent of people with Sjogren’s have these antibodies, a negative outcome does not mean that the disease can be ruled out. This also creates confusion during the initial diagnosis.

Ophthalmological tests: The Rose Bengal, Lissamine green, and Schirmer eye tests can be conducted by an ophthalmologist. In order to expose dry areas of the eye, dyes and blotting paper are used.

Salivary flow rate: over 5 minutes, the physician calculates the weight of the saliva produced in a cup. This can mean Sjogren’s, if insufficient saliva is made.

Sialogram: In the parotid glands, a dye is injected. To assess how much saliva flows into the patient’s mouth, an X-ray is then taken. Increasingly, this practice is used less.

Salivary scintigraphy: To measure the activity of salivary glands, a radioactive isotope is injected and monitored by imaging.

X-ray or CT-scan of the chest: This measures the presence of inflammation that can be caused by Sjogren’s in the lungs.

Nerve fiber stain skin biopsy: This decides the presence of small fiber neuropathy (SFN).

Urine testing: The urine is sampled and analyzed to determine whether it has damaged the kidneys.

Rash

Purpura, a type of vasculitis
Purpura, a type of vasculitis, may occur.

People with Sjogren’s, especially after spending time in the sun, may be more prone to a rash.

Problems with the skin that occur with the condition can appear as:

  • xerosis, or dry, rough skin
  • small “blood spots” or purpura on the lower legs due to vasculitis, or inflammation of the blood vessels
  • vasculitic skin lesions that may appear as weals, lumps, blisters, or ulcers
  • red, ring-shaped lesions with a pale area in the middle, known as annular erythema

It can help avoid dry skin by using a good moisturizer. The doctor can prescribe drugs to suppress the immune system, such as cyclophosphamide, if vasculitis occurs.

Complications

Sjogren’s comes with serious complications if left untreated, including:

  • an increased risk of lymphoma and multiple myeloma
  • oral yeast infections
  • dental cavities
  • vision problems
  • inflammation leading to bronchitispneumonia, and other lung problems
  • kidney function problems
  • autoimmune hepatitis or cirrhosis in the liver
  • giving birth to a baby with heart problems or lupus
  • peripheral neuropathy
  • interstitial cystitis of the bladder

It is important to seek treatment for Sjogren’s as early as possible to prevent these.

Causes

Sjogren’s is caused by white blood cells.
Sjogren’s is caused by white blood cells mistakenly attacking moisture-producing glands.

Sjogren’s causes remain largely unidentified.

Studies have shown that the disease can be caused by a viral or bacterial infection, but that the underlying cause is mostly genetic and environmental. Also involved in the onset of Sjogren’s is the nervous system and the endocrine, or hormone-producing, system.

An environmental factor, such as infection with hepatitis C or the Epstein-Barr virus, can change the immune system and cause immune problems later on.

As the majority of people who develop Sjogren’s are female, one theory is that a significant role is played by estrogen, a female hormone. However this is still not understood.

The most common time for a diagnosis to be made of Sjogren’s is menopause. Some studies theorize that Sjogren’s is covered by estrogen, and dropping hormone levels can alter immune function and cause the condition.

For Sjogren, there’s no cure. An individual with the condition can however, maintain a good quality of life by successfully managing the dryness of the affected regions and treating any affected organs.

A variety of clinical research projects are currently in progress focused on discovering new treatments for Sjogren’s.

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