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What organs can sjogrens affect?

Sjögren’s Syndrome

Sjögren’s syndrome is a chronic, autoimmune disease. It can occur alone or with other autoimmune conditions like rheumatoid arthritis (RA) or lupus. Sjögren’s syndrome presents with dry eyes and dry mouth. Inflammation of the tear ducts and saliva glands cause dryness and irritation. Other common symptoms include joint and muscle pain, fatigue, and rashes. Less commonly, it can affect internal organs such as the lungs, gastrointestinal tract, kidneys, and nervous system. Sjögren’s syndrome affects women about 10 times more often than men and usually occurs between 45 and 55. Symptoms can range, but patients can often lead a normal life. Rarely, it can cause complications like an increased risk of lymphoma, a type of cancer.

What Are the Signs/Symptoms?

Dry eyes and dry mouth are common symptoms of Sjögren’s syndrome. The eyes may turn red, burn or feel gritty like sand in your eyes. It may be hard to eat dry foods or swallow because of lack of saliva. Glands of the neck or face may swell up. Other symptoms include: dryness of skin, vagina, nasal passages and throat; heartburn; joint and muscle pains; fatigue; and rashes. Because of chronic dryness, patients may be at risk for eye infections or cornea damage, tooth decay or gum disease, and vaginal yeast infections.

Diagnosing Sjögren’s syndrome includes a physical exam, blood tests and sometimes a biopsy. An eye exam by the ophthamologist may be necessary to evaluate dry eyes. A rheumatologist can do blood tests for anti-nuclear antibodies (ANA), anti-SSA (Ro) and SSB (La) antibodies, and rheumatoid factor (RF), or obtain a salivary gland biopsy to make a diagnosis.

What Are Common Treatments?

Treatments for Sjögren’s syndrome relieve symptoms of dryness and prevent problems like infections or dental disease. Treatments for dry eyes include artificial tears or eye gels; eye drops such as cyclosporine (Restasis) to increase tear production. For dry mouth, sips of water or chewing gum may help, but some patients need prescription drugs like pilocarpine (Salagen) or cevimeline (Evoxac) to increase saliva production. Patients with reflux may use proton-pump inhibitors or H2 blockers. Depending on the symptoms and organ system involved, some people with Sjögren’s syndrome may need hydroxychloroquine, corticosteroids (Deltasone), methotrexate (Rheumatrex), azathioprine(Imuran), mycophenolate (Cellcept), rituximab (Rituxan) or cyclophosphamide (Cytoxan) in life threatening cases.

Living with Sjögren’s Syndrome

Here are some tips:

  • Get regular dental check-ups to prevent tooth decay and cavities.
  • Get regular eye exams with an ophthalmologist to check for cornea damage or infections.
  • Get regular follow up with a rheumatologist who can treat symptoms and monitor for complications.

Updated February 2023 by Kristen Lee, MD, and reviewed by the Sjögren’s Foundation and the American College of Rheumatology Committee on Communications and Marketing.

This information is provided for general education only. Individuals should consult a qualified health care provider for professional medical advice, diagnosis and treatment of a medical or health condition.

Sjögren’s Syndrome

Sjögren’s syndrome is a disease in which the body’s defense system (immune system) attacks healthy tissues causing dryness of the mouth, eyes and other membranes of the body that secrete mucous. It is often found along with rheumatic disorders such as rheumatoid arthritis, scleroderma and lupus.

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Symptoms

Sjögren’s syndrome may affect only the eyes or only the mouth or it may be more general. One out of three people who have Sjögren’s syndrome also have arthritis. A person may have signs of a rheumatic disease, but not have the dry eyes or mouth associated with some forms of Sjögren’s.

The disease damages the glands that supply saliva to the mouth and tears to the eyes. This causes the cornea of the eye and the tissues around the eye to dry out. The eyes may feel scratchy or irritated. In advanced cases, the cornea may be damaged, impairing vision.

Persons with Sjögren’s syndrome have little or no saliva. This causes a dry mouth and lips. It also makes chewing and swallowing difficult and promotes tooth decay. The disease may also affect a person’s ability to taste or smell food.

Other parts of the body where there are mucous membranes also begin to dry out. This includes the nose, throat, lungs, vulva and vagina. Drying in the lungs can result in lung infections or pneumonia. There may be a loss of hair on the body.

Other symptoms may include:

  • Chronic diseases of the liver, gall bladder and pancreas
  • Loss of the ability of the nerves to feel sensation
  • Kidney disorders

Sjögren’s syndrome is more common than lupus but less common than rheumatoid arthritis. While its cause is not known, there may be a genetic factor.

A person who has Sjögren’s syndrome has a 44 times higher risk of lymphoma compared to other people. Additionally, such individuals are at higher risk of Waldenström’s macroglobulinemia.

Diagnosis

To test whether a person has Sjögren’s syndrome a doctor will do the following tests:

  • Schirmer test. This test measures the amount of tears the eye forms when irritated by a filter paper strip placed under each lower eyelid
  • Putting a drop of rose of Bengal solution, which stains the areas of the eye that absorb it, can also help identify the dryness of the eye that is characteristic of Sjögren’s
  • Tests to measure the flow of saliva in the mouth
  • A biopsy of salivary glands in the mouth for examination under a microscope
  • Blood tests to measure whether the body’s immune system is reacting

Treatment

Currently, treatment focuses on any related rheumatic disease that may be present and treating the symptoms of Sjögren’s syndrome. Treatment may include:

  • Sipping fluids throughout the day
  • Chewing sugarless gum
  • Using a saliva substitute as a mouthwash
  • Avoiding antihistamines or other drugs that cause mucous membranes to dry up
  • Being careful about regular mouth and tooth care and regular visits to the dentist
  • A drug, pilocarpine, may be used to make the gland produce more saliva if they haven’t been severely shrunken by the disease
  • Pain relievers may be prescribed when needed
  • Using eye drops
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© 2000-2022 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional’s instructions.

Sjögren’s Syndrome Dry Mouth and Eyes

Sjögren’s syndrome can cause various symptoms. The most common ones are dry eyes and dry mouth. These symptoms are due to lack of secretions from glands in the body. In severe cases the lungs, kidneys, nervous system and lymph glands can be affected. Treatment is mainly directed at symptom control.

Sjögren’s Syndrome

Dry Mouth and Eyes
In this article
  • What is Sjögren’s syndrome?
  • How common is Sjögren’s syndrome?
  • What are the symptoms of Sjögren’s syndrome?
  • Are there any complications of Sjögren’s syndrome?
  • Other problems associated with Sjögren’s syndrome
  • How is Sjögren’s syndrome diagnosed?
  • What is the treatmentfor Sjögren’s syndrome?
  • What is the outlook (prognosis) of Sjögren’s syndrome?

What is Sjögren’s syndrome?

Sjögren’s syndrome most commonly causes dry eyes and mouth. It can also affect other organs including lungs, kidneys, skin and the nervous system. It was first described in 1933 by a Swedish eye specialist (ophthalmologist) called Henrik Sjögren.

Sjögren’s syndrome is an autoimmune disease. Normally, our body makes antibodies to fight infections — for example, when we catch a cold or have a sore throat. These antibodies help to kill the cells of the germs (bacteria or viruses) causing the infection. With autoimmune conditions the body makes similar antibodies (autoantibodies) and the immune system attacks its own cells. The cause of this is uncertain but is thought to be a combination of genes and factors in the environment such as infection.

In Sjögren’s syndrome, these autoantibodies attack the cells of certain glands. The effect is that these glands cannot release their normal secretions. This means that the symptoms of Sjögren’s syndrome are mainly due to dryness and lack of gland secretions.

Primary Sjögren’s syndrome

This describes Sjögren’s syndrome which occurs by itself. About one in a thousand people have this.

Secondary Sjögren’s syndrome

This describes Sjögren’s syndrome which occurs in association with another autoimmune disease such as rheumatoid arthritis or systemic lupus erythematosus.

How common is Sjögren’s syndrome?

It is thought there are about half a million people in the UK with Sjögren’s syndrome, although not all of them seek medical help for their symptoms. Most people who have the disease are women. People usually first start noticing symptoms when they are in their 20s or 40s, but many people have symptoms for a number of years before the diagnosis of Sjögren’s syndrome is made.

What are the symptoms of Sjögren’s syndrome?

The two symptoms that everyone with Sjögren’s syndrome will notice are:

  • Dry mouth (xerostomia).
  • Dry eyes (xerophthalmia). Eyes may feel gritty and uncomfortable.

The dryness of the eyes, mouth and other body parts is known as sicca syndrome. As well as Sjögren’s syndrome, sicca syndrome can also be caused by radiotherapy treatment and other diseases such as sarcoidosis and haemochromatosis.

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Dry mouth

This can lead to:

  • Swallowing problems and the feeling of something getting stuck in the throat on swallowing (dysphagia).
  • Thrush (a yeast infection) in the mouth.
  • Loss of taste.
  • Tooth decay and gum disease (gingivitis). Saliva contains anti-infective agents, so when saliva production is reduced, infection in the mouth is more likely.

Dryness in other body parts

  • Vaginal dryness can cause discomfort when having sex (dyspareunia).
  • Dryness of the upper airways (trachea and bronchus) can lead to a dry cough and chest infections.
  • Dry skin can occur.

Other symptoms

You may notice the following occurring:

  • Tiredness.
  • Muscle aches and aching joints. Sjögren’s syndrome often affects people who have other autoimmune diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus.
  • Swelling of the salivary glands, including the parotid glands (located in both cheek areas, just in front of the ears) and those under the jaw and in the neck area.

Are there any complications of Sjögren’s syndrome?

Some people who have Sjögren’s syndrome develop complications such as:

  • Infection of the salivary glands.
  • Corneal ulcers: dry eyes can lead to infection and the development of ulcers on the surface of the eyes. If not treated, this can lead to loss of vision.
  • Pancreatitis: this is inflammation of the pancreas gland, characterised by severe pain in the upper part of the stomach (abdomen).
  • Peripheral neuropathy: this causes loss of sensation in fingers, hands, arms, toes, feet, and legs.
  • Cranial neuropathy: this causes loss of sensation in parts of the face.
  • Kidney problems: Sjögren’s syndrome can progress to affect the kidneys. It can cause kidney inflammation, disruption in body fluid balance, kidney stones and, if untreated, loss of kidney function.
  • Pseudolymphoma: 1 in 10 people with Sjögren’s syndrome can develop a condition called pseudolymphoma. This causes enlargement of the spleen (an organ in the left upper abdomen that helps the body fight infection and get rid of old red blood cells). It also causes enlargement of the lymph glands in other parts of the body.
  • Non-Hodgkin’s lymphoma:
    • in some people who develop pseudolymphoma, the pseudolymphoma can progress to a lymphoma, usually non-Hodgkin’s lymphoma.
    • The first symptom noticed with non-Hodgkin’s lymphoma is usually swelling of the lymph glands, particularly in the neck.
    • If you are diagnosed with Sjögren’s syndrome, you should watch for the development of any lumps or swellings in your neck, groin or under your arms and report anything abnormal to your doctor.

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    Other problems associated with Sjögren’s syndrome

    • Recurrent miscarriage: Sjögren’s syndrome can cause recurrent miscarriage. Recurrent miscarriage is the term used when a woman has three or more miscarriages in a row. This is because of a link between Sjögren’s syndrome and a condition called antiphospholipid syndrome.
    • Medication reactions: people with Sjögren’s syndrome may be more prone to developing side-effects when they take certain medicines — for example, antibiotic medicines.
    • Raynaud’s phenomenon: the extremities of the body, usually the fingers and toes, change colour and may become painful, usually due to exposure to the cold.

    How is Sjögren’s syndrome diagnosed?

    There are a number of investigations that your doctor may do to help diagnose Sjögren’s syndrome.

    • The Schirmer tear test: this measures the amount of tears that you form. A piece of filter paper is placed under the lower lid of your eye and left for five minutes. In Sjögren’s syndrome, tear production is reduced.
    • Slit-lamp examination: a dye is used to stain the eye temporarily. The doctor will look into your eyes, using a special lamp, to see if there is reduced tear production or eye damage.
    • Salivary gland biopsy: one of the tiny salivary glands may be removed from your lower lip for examination. This is a simple procedure and can be done using an injection of local anaesthetic. Special dyes and staining are then used in the laboratory to look for signs of Sjögren’s syndrome in this gland.
    • Saliva collection: you may be asked to collect the saliva that you make over 10 minutes. A reduced volume suggests Sjögren’s syndrome.
    • Blood tests: your doctor may also want you to have some blood tests that may suggest inflammation or signs of an autoimmune disease.

    Some other tests may be arranged if the diagnosis is not clear or your doctor suspects a complication. For example:

    • An ultrasound scan to look at your internal organs and lymph glands.
    • Tests on your urine to look at your kidney function.

    What is the treatmentfor Sjögren’s syndrome?

    There is currently no cure for Sjögren’s syndrome. Treatment is aimed at controlling and relieving symptoms such as the reduction in tears and saliva.

    You may be referred to a number of different specialists when you are diagnosed with Sjögren’s syndrome, depending on the parts of your body that it affects. Joint specialists (rheumatologists) are the main doctors who have specialist knowledge of Sjögren’s syndrome. This is because of the association of Sjögren’s syndrome with other diseases that affect the joints, such as rheumatoid arthritis and systemic lupus erythematosus. However, you may also be referred to a dentist, an eye specialist, a lung specialist or a kidney specialist. Your GP will continue to provide support for you and will usually prescribe your medication under the guidance of these other specialists.

    Treatment of dry eyes

    • Avoid situations that make dry eye symptoms worse. These may include:
      • Low humidity and air conditioning.
      • Wind.
      • Dust and smoke (so, if appropriate, try to stop smoking if you are a smoker).
      • Contact lenses.
      • Prolonged reading or staring at a computer screen or television. This makes us blink less often so our eyes don’t stay as moist.

      Treatment of dry mouth and related symptoms

      • General measures that can help include:
        • Sipping water throughout the day.
        • Keeping your teeth, gums and mouth as clean and healthy as possible. Brush your teeth regularly, use dental floss and a mouth wash.
        • Visiting your dentist regularly.
        • Using Vaseline® for dry, cracked lips.
        • Chewing sugar-free chewing gum.

        Treatment of other symptoms

        • Moisturisers and special bath additives can be used for dry skin.
        • Lubricants may be needed when you have sex.
        • Non-steroidal anti-inflammatory drugs (NSAIDs) can be taken to help joint and muscle pains.

        Treatment of complications

        If Sjögren’s syndrome progresses to involve organs such as the skin, lungs, kidneys and lymph glands, you may need to take some other medication. Such medication may include:

        • Steroids: these are tablets taken by mouth that help to reduce inflammation. They may be prescribed if your symptoms are particularly bad.
        • Immunosuppressive agents: these are medicines that damp down the abnormal antibody production in Sjögren’s syndrome. Names include methotrexate, penicillamine and hydroxychloroquine. As with steroids, they are reserved for severe cases as they do have side-effects and you will need close monitoring with blood tests while you are taking them. You may be prescribed one of these medicines if your kidneys or lungs are affected, or if you develop pseudolymphoma.
        • A medicine called rituximab may be used if there is a poor response to treatment with steroids and other immunosuppressive agents.

        What is the outlook (prognosis) of Sjögren’s syndrome?

        Sjögren’s syndrome is not usually life-threatening. Some people may only notice mild symptoms such as mildly dry eyes and a mildly dry mouth. Other people develop more irritating and disabling symptoms affecting their eyes, mouth, vision and eating and can also have uncomfortable joint pains and tiredness.

        Sometimes symptoms can go away for long periods of time (go into remission). Some people develop more serious problems such as the kidney and lung problems described above.

        Some people with Sjögren’s syndrome develop another autoimmune disorder such as rheumatoid arthritis, systemic lupus erythematosus or polymyositis.

        As mentioned above, a small number of people with Sjögren’s syndrome develop lymphoma, most commonly non-Hodgkin’s lymphoma. This lymphoma can usually be treated and your doctor will review you regularly to look for signs of this.

        Further reading and references

        • The British Society for Rheumatology guideline for the management of adults with primary Sjogren’s Syndrome ; British Society for Rheumatology (2017)
        • Cafaro G, Bursi R, Chatzis LG, et al ; One year in review 2021: Sjogren’s syndrome. Clin Exp Rheumatol. 2021 Nov-Dec39 Suppl 133(6):3-13. doi: 10.55563/clinexprheumatol/eojaol. Epub 2021 Dec 1.
        • Stefanski AL, Tomiak C, Pleyer U, et al ; The Diagnosis and Treatment of Sjogren’s Syndrome. Dtsch Arztebl Int. 2017 May 26114(20):354-361. doi: 10.3238/arztebl.2017.0354.
        • Jonsson R, Brokstad KA, Jonsson MV, et al ; Current concepts on Sjogren’s syndrome — classification criteria and biomarkers. Eur J Oral Sci. 2018 Oct126 Suppl 1:37-48. doi: 10.1111/eos.12536.
        • Baer AN, Walitt B ; Update on Sjogren Syndrome and Other Causes of Sicca in Older Adults. Rheum Dis Clin North Am. 2018 Aug44(3):419-436. doi: 10.1016/j.rdc.2018.03.002.
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