Sjogren's Syndrome

Sjogren's Syndrome: Overview

Alternative names: Sjögren's (pronounced SHOW-grins), SS

Sjogren's Syndrome is a chronic autoimmune disease in which the body's immune system attacks moisture-producing glands and causes dryness in the mouth and eyes.  Other parts of the body can be affected as well, resulting in a wide range of possible symptoms.

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Sjogren's syndrome is classified as either primary or secondary.  Primary Sjogren's occurs by itself, and secondary Sjogren's occurs with another disease.  Both are systemic disorders (affecting the whole body), although the symptoms in the primary form are more restricted.

Location of the three main saliva glands under tongue, jaw, ears
Major salivary glands: Sublingual gland under tongue; submandibular glands below jawbone; parotid glands in front of earlobes

In cases of primary Sjogren's syndrome, the doctor can trace the symptoms to problems with the tear and saliva glands.  These patients are likely to have different antibodies circulating in their blood compared to people with the secondary form.  These antibodies are called SS-A and SS-B; people with primary Sjogren's are also more likely to have antinuclear antibodies (ANAs) in their blood.  ANAs are autoantibodies that are directed against the body.

In cases of secondary Sjogren's syndrome, the patient will have had an autoimmune disease such as rheumatoid arthritis or lupus before Sjogren's developed.  People with this type tend to have more health problems because they have two diseases, and they are also less likely to have the antibodies associated with primary Sjogren's.

Incidence; Causes and Development

Sjogren's Syndrome is believed to affect up to 4 million people in the U.S., most (90%) of them women.  It can occur at any age, but is usually diagnosed in those aged 40-60; it can affect people of all races and ethnic backgrounds.

The disease-fighting cells of the immune system attack the glands that produce tears and saliva (the lacrimal and salivary glands).  Sjogren's can affect other glands too, such as those in the stomach, pancreas and intestines, and can cause dryness in other places that need moisture, such as the nose, throat, airways, vagina and skin.

Researchers believe Sjogren's syndrome is caused by a combination of genetic and environmental factors.  Having one of the associated genes will not cause a person to develop the disease without some sort of trigger to activate the immune system, for example a viral or bacterial infection.

Signs and Symptoms

Although no one knows exactly how damage occurs to the tear and saliva glands, they are longer able to produce tears and saliva, resulting in the main symptoms of Sjogren's syndrome:

  • Dry eyes – The eyes may be red and burn and itch.  People say it feels like they have sand in their eyes.  Also, vision may be blurry, and bright light, especially fluorescent lighting, may be difficult to tolerate.
  • Dry mouth – Dry mouth feels like a mouthful of cotton.  It's difficult to swallow, speak, or taste.  Sense of smell can change, and a dry cough may develop.  Also, because of the protective effects of saliva, dry mouth increases chances of developing cavities and mouth infections.

When Sjögren's syndrome affects other parts of the body, the condition is called extraglandular involvement because the problems extend beyond the tear and salivary glands.

About half of those with Sjogren's have dry skin.  Some experience only itching, possibly severe.  Others develop cracked, split skin that can easily become infected.  Infection is a risk for people with itchy skin, too, particularly if they scratch vigorously.  The skin may darken in infected areas, but it returns to normal when the infection clears up and the scratching stops.

Some Sjogren's patients, particularly those who also have lupus, are sensitive to sunlight and can get painful burns from even a little sun exposure, such as through a window.

Other symptoms
Other symptoms of extraglandular involvement include:

  • Skin rashes
  • Thyroid problems
  • Joint and muscle pain
  • Recurrent sinus infections
  • Lung and airway problems, including:

    • Persistent dry cough (in about half of cases)
    • Nosebleeds (in about 1-in-3 cases)
    • Nasal crusting/scabs (in about 1-in-5 cases)
    • Hoarseness due to dryness or a thick mucus coating the vocal cords (in about 1-in-3 cases)
    • Pneumonia (various types)
    • Chronic Obstructive Pulmonary Disease (COPD) (in about 2-in-5 cases)
    • Cystic lung disease (in about 1-in-10 primary cases)
  • Vaginal dryness is common in affected women and painful intercourse is the most common complaint
  • Neurological symptoms

    • Peripheral neuropathy – numbness and tingling in the extremities
    • Autonomic neuropathy – damage to nerves that help regulate the coordination of heartbeat, respiration, and gastric motility
    • Trigeminal neuralgia – numbness or burning of the face
    • Glossopharyngeal neuralgia – pain in the back of the throat
    • Mononeuritis multiplex – weakness or paralysis of various muscles
  • Kidney problems (tubular interstitial nephritis, renal tubular acidosis – a risk factor for kidney stones, glomerular disease)
  • Gastrointestinal symptoms

  • Inflammation of blood vessels (vasculitis)
  • Connective tissue disorders
  • Debilitating fatigue

Diagnosis and Tests

The doctor will first take a detailed medical history, which includes asking questions about general health, symptoms, family medical history, alcohol consumption, smoking, or use of drugs or medications.  The doctor will also do a complete physical exam to check for signs of Sjogren's.

First, the doctor will want to check the eyes and mouth to see whether Sjogren's is causing the symptoms and how severe the problem is.  Because there are many possible causes of dry eyes and dry mouth, the doctor will take these into account.

One is generally considered to have definite Sjogren's if one has dry eyes, dry mouth, and a positive lip biopsy.  The doctor may decide to perform additional tests to see whether other parts of the body are affected, and whether various antibodies are found in the blood.

A woman undergoing Schirmer's test for dry eyes
Schirmer's test uses dye-containing eye drops and a paper test strip to determine if the eye is producing enough tears

Common eye and mouth tests are:

  • Schirmer test – This test measures tears to see how the lacrimal gland is working.  It can be done in two ways: In Schirmer I, the doctor puts thin paper strips under the lower eyelids and measures the amount of wetness on the paper after 5 minutes.  People with Sjogren's usually produce less than 8 millimeters of tears.  The Schirmer II test is similar, but the doctor uses a cotton swab to stimulate a tear reflex inside the nose.
  • Staining with vital dyes (rose bengal or lissamine green) – The tests show how much damage dryness has done to the surface of the eye.  The doctor puts a drop of a liquid containing a dye into the lower eye lid.  These drops stain on the surface of the eye, highlighting any areas of injury.
  • Slit lamp examination – This test shows how severe the dryness is and whether the outside of the eye is inflamed.  An ophthalmologist (eye specialist) uses equipment that magnifies to carefully examine the eye.
  • Mouth exam – The doctor will look in the mouth for signs of dryness and to see whether any of the major salivary glands are swollen.  Signs of dryness include a dry, sticky mouth; cavities; thick saliva, or none at all; a smooth look to the tongue; redness in the mouth; dry, cracked lips; and sores at the corners of the mouth.  The doctor might also try to get a sample of saliva to see how much the glands are producing and to check its quality.
  • Salivary gland biopsy of the lip – This test is the best way to find out whether dry mouth is caused by Sjogren's syndrome.  The doctor removes tiny minor salivary glands from the inside of the lower lip and examines them under the microscope.  If the glands contain lymphocytes in a particular pattern, the test is positive for Sjogren's syndrome.
Antibodies seen while looking down inside of a blood vessel
The diagnostic process can include blood tests that look for specific antibodies

Other tests may include:

  • Routine blood tests – The doctor will take blood samples to check blood count and blood sugar level, and to see how the liver and kidneys are working.
  • Immunological tests – These blood tests check for antibodies commonly found in the blood of people with Sjogren's syndrome.  For example:

    • Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland.  Antithyroid antibodies cause thyroiditis (inflammation of the thyroid), a common problem in people with Sjogren's.
    • Immunoglobulins and gamma globulins are antibodies that everyone has in their blood, but people with Sjogren's usually have too many of them.
    • Rheumatoid factors (RFs) are found in the blood of people with rheumatoid arthritis, as well as in people with Sjogren's.  Substances known as cryoglobulins may be detected; these indicate risk of lymphoma.
    • Similarly, the presence of antinuclear antibodies (ANAs) can indicate an autoimmune disorder, including Sjogren's.
    • Sjogren's antibodies, called SS-A (or SS-Ro) and SS-B (or SS-La), are specific antinuclear antibodies common in people with Sjogren's.  However, you can have Sjogren's without having these ANAs.
  • Chest X-ray – Sjogren's can cause inflammation in the lungs, so the doctor may want to take an X-ray to check them.
  • Urinalysis – The doctor will probably test a sample of your urine to see how well the kidneys are working.

Treatment and Prevention

General Tips for Eye Care

  • Don't use artificial tears that irritate your eyes – try another brand or preparation.
  • Nonpreserved drops may be more comfortable.
  • Blink several times a minute while reading or working on the computer.
  • Protect your eyes from drafts, breezes, and wind.
  • Put a humidifier in the rooms where you spend the most time, including the bedroom, or install a humidifier in your heating and air conditioning unit.
  • Don't smoke and stay out of smoky rooms.
  • Apply mascara only to the tips of your lashes so it doesn't get in your eyes.  If you use eyeliner or eye shadow, put it only on the skin above your lashes, not on the sensitive skin under your lashes, close to your eyes.
  • Ask your doctor whether any of your medications contribute to dryness and, if so, how to reduce that effect.

General Tips for Dry Mouth
If your salivary glands still produce some saliva, you can stimulate them to make more by chewing gum or sucking on hard candy.  However, gum and candy must be sugar free because dry mouth makes you extremely prone to cavities.  Take sips of water or another sugar free drink often throughout the day to wet your mouth, especially when you are eating or talking.  Note that you should take sips of water – drinking large amounts of liquid throughout the day will not make your mouth any less dry.  It will only make you urinate more often and may strip your mouth of mucus, causing even more dryness.  You can soothe dry, cracked lips by using oil- or petroleum-based lip balm or lipstick.  If your mouth hurts, the doctor may give you medicine in a mouth rinse, ointment, or gel to apply to the sore areas to control pain and inflammation.

If you produce very little saliva or none at all, your doctor might recommend a saliva substitute.  These products mimic some of the properties of saliva, which means they make the mouth feel wet, and if they contain fluoride, they can help prevent cavities.  Gel-based saliva substitutes tend to give the longest relief, but all saliva products are limited since you eventually swallow them.

At least two drugs that stimulate the salivary glands to produce saliva are available.  These are pilocarpine and cevimeline.  The effects last for a few hours, and you can take them three or four times a day.  However, they are not suitable for everyone, so talk to your doctor about whether they might help you.

People with dry mouth can easily get mouth infections.  Candidiasis, a fungal mouth infection, is one of the most commonly seen in people with Sjogren's.  It most often shows up as white patches inside the mouth that you can scrape off, or as red, burning areas in the mouth.  Candidiasis is treated with antifungal drugs.  Various viruses and bacteria can also cause infections; they're treated with the appropriate antiviral or antibiotic medicines.

Oral Hygiene is Important
Natural saliva contains substances that rid the mouth of the bacteria that cause cavities and mouth infections, so good oral hygiene is extremely important when you have dry mouth.  Here's what you can do to prevent cavities and infections:

  • Visit a dentist at least three times a year to have your teeth examined and cleaned.
  • Rinse your mouth with water several times a day.  Don't use mouthwash that contains alcohol because alcohol is drying.
  • Gently brush your teeth, gums, and tongue after each meal and before bedtime.  Nonfoaming toothpaste is less drying.
  • Floss your teeth every day.
  • Avoid sugar.  That means choosing sugar-free gum, candy, and soda.  If you do eat or drink sugary foods, brush your teeth immediately afterward.
  • Look at your mouth every day to check for redness or sores.  See a dentist right away if you notice anything unusual or have any mouth pain or bleeding.
  • Ask your dentist whether you need to have a protective varnish put on your teeth to protect the enamel.

To treat dry skin, apply heavy moisturizing creams and ointments three or four times a day to trap moisture in the skin.  Lotions, which are lighter than creams and ointments, aren't recommended because they evaporate quickly and can contribute to dry skin.  Also, doctors suggest that you take only a short shower (less than 5 minutes), use a moisturizing soap, pat your skin almost dry, and then cover it with a cream or ointment.  If you take baths, it's a good idea to soak for 10 to 15 minutes to give your skin time to absorb moisture.  Having a humidifier in the bedroom can help hydrate your skin, too.  If these steps don't help the itching, your doctor might recommend that you use a skin cream or ointment containing steroids.

Those who are sensitive to sunlight should wear sunscreen (at least SPF 15) whenever they go outdoors, and try to avoid being in the sun for long periods of time.

Vaginal Dryness
A vaginal moisturizer helps retain moisture, and a vaginal lubricant can make intercourse more comfortable.  Vaginal moisturizers attract liquid to the dry tissues and are designed for regular use.  Vaginal lubricants should be used only for intercourse – they don't moisturize.  Oil-based lubricants, such as petroleum jelly, trap moisture and can cause sores and hinder the vagina's natural cleaning process.  A water-soluble lubricant is better.

Regular skin creams and ointments relieve dry skin on the outer surface of the vagina (the vulva).

Lung Problems
People with Sjogren's also tend to have lung problems caused by inflammation, such as bronchitis (affecting the bronchial tubes), tracheobronchitis (affecting the windpipe and bronchial tubes), and laryngotracheobronchitis (affecting the voice box, windpipe, and bronchial tubes).  Depending on your condition, the doctor may recommend using a humidifier, taking medicines to open the bronchial tubes, or taking corticosteroids to relieve inflammation.  Pleurisy is inflammation of the lining of the lungs and is treated with corticosteroids and nonsteroidal antiinflammatory drugs.

Prognosis; Complications

The outcome of Sjogren's Syndrome is almost impossible to predict.  Although the disease rarely shortens a patient's life span, it can have many life-changing aspects that vary from mild to devastating.

Protect Your Voice. People with Sjögren's can develop hoarseness if their vocal cords become inflamed as part of the disease or become irritated from throat dryness or coughing.  To prevent further strain on your vocal cords, try not to clear your throat before speaking.  Instead, take a sip of water, chew gum, or suck on candy.  Or else make an "h" sound, hum, or laugh to gently bring the vocal cords together so you can get sound out.  Clearing your throat does the same thing, but it's hard on the vocal cords, and you want to avoid irritating them further.

Kidney Problems. The kidneys filter waste products from the blood and remove them from the body through urine.  The most common kidney problem in people with Sjogren's is interstitial nephritis, or inflammation of the tissue around the kidney's filters, which can occur even before dry eyes and dry mouth.  Inflammation of the filters themselves, called glomerulonephritis, is less common.  Some people develop renal tubular acidosis, which means they can't get rid of certain acids through urine.  The amount of potassium in their blood drops, causing an imbalance in blood chemicals that can affect the heart, muscles, and nerves.

Often, doctors do not treat these problems unless they start to affect kidney function or cause other health problems.  However, they keep a close eye on the problem through regular exams, and will prescribe medicines called alkaline agents to balance blood chemicals when necessary.  Corticosteroids or immunosuppressants are used to treat more severe cases.

Nerve Problems. People with Sjogren's syndrome can have nerve problems.  When they do, the problem usually involves the peripheral nervous system (PNS), which contains the nerves that control sensation and movement.  Involvement of the PNS is increasingly being recognized.  Carpal tunnel syndrome, peripheral neuropathy, and cranial neuropathy are examples of peripheral nervous system disorders that occur in people with Sjogrens.  In carpal tunnel syndrome, inflamed tissue in the forearm presses against the median nerve, causing pain, numbness, tingling, and sometimes muscle weakness in the thumb and index and middle fingers.  In peripheral neuropathy, an immune attack damages nerves in the legs or arms, causing the same symptoms there.  (Sometimes nerves are damaged because inflamed blood vessels cut off their blood supply.)  In cranial neuropathy, nerve damage causes face pain; loss of feeling in the face, tongue, eyes, ears, or throat; and loss of taste and smell.

Nerve problems are treated with medicines to control pain and, if necessary, with steroids or other drugs to control inflammation.

Digestive Problems. Inflammation in the esophagus, stomach, pancreas, and liver can cause problems like painful swallowing, heartburn, abdominal pain and swelling, loss of appetite, diarrhea, and weight loss.  It can also cause hepatitis (inflammation of the liver) and cirrhosis (hardening of the liver).  Sjogren's is closely linked to a liver disease called primary biliary cirrhosis (PBC), which causes itching, fatigue, and, eventually, cirrhosis.  Many patients with PBC have Sjogren's.

Treatment varies, depending on the problem, but may include pain medicine, anti-inflammatory drugs, steroids, and immunosuppressants.

Connective Tissue Disorders. Connective tissue is the framework of the body that supports organs and tissues.  Examples are joints, muscles, bones, skin, blood vessel walls, and the lining of internal organs.  Many connective tissue disorders are autoimmune diseases, and several are common among people with Sjogren's:

  • Polymyositis is an inflammation of the muscles that causes weakness and pain, difficulty moving, and, in some cases, problems breathing and swallowing.  If the skin is inflamed too, it's called dermatomyositis.  The disease is treated with corticosteroids and immunosuppressants.
  • In Raynaud's phenomenon, blood vessels in the hands, arms, feet, and legs constrict (narrow) when exposed to cold.  The result is pain, tingling, and numbness.  When vessels constrict, fingers turn white.  Shortly after that, they turn blue because of blood that remained in the tissue pools.  When new blood rushes in, the fingers turn red.  The problem is treated with medicines that dilate blood vessels.  Raynaud's phenomenon usually occurs before dryness of the eyes or mouth.
  • Rheumatoid arthritis (RA) is severe inflammation of the joints that can eventually deform the surrounding bones (fingers, hands, knees, etc.).  RA can also damage muscles, blood vessels, and major organs.  Treatment depends on the severity of the pain and swelling and which body parts are involved.  It may include physical therapy, aspirin, rest, nonsteroidal anti-inflammatory agents, steroids, or immunosuppressants.
  • Scleroderma causes the body to accumulate too much collagen, a protein commonly found in the skin.  The result is thick, tight skin and damage to muscles, joints, and internal organs such as the esophagus, intestines, lungs, heart, kidneys, and blood vessels.  Treatment is aimed at relieving pain and includes drugs, skin softeners, and physical therapy.
  • Systemic lupus erythematosus (SLE) causes joint and muscle pain, weakness, skin rashes, and, in more severe cases, heart, lung, kidney, and nervous system problems.  As with RA, treatment for SLE depends on the symptoms and may include aspirin, rest, steroids, and anti-inflammatory and other drugs, as well as dialysis and high blood pressure medicine.
  • Vasculitis is an inflammation of the blood vessels, which then become scarred and too narrow for blood to get through to reach the organs.  In people with Sjogren's, vasculitis tends to occur in those who also have Raynaud's phenomenon and lung and liver problems.
  • Autoimmune thyroid disorders are common with Sjogren's.  They can appear as either the overactive thyroid of Graves' disease or the underactive thyroid of Hashimoto's.  Nearly half of the people with autoimmune thyroid disorder also have Sjogren's, and many people with Sjogren's show evidence of thyroid disease.

Signs, symptoms & indicators of Sjogren's Syndrome:

Lab Values - Chemistries

Symptoms - Head - Eyes/Ocular

(Very) dry eyes

"Dry eye" is a symptom of certain autoimmune diseases like rheumatoid arthritis and Sjogren's syndrome, in which the body's immune system mistakenly attacks the tear ducts and saliva glands.

(Very) watery eyes

"Dry eye" is a symptom of certain autoimmune diseases like rheumatoid arthritis and Sjogren's syndrome, in which the body's immune system mistakenly attacks the tear ducts and saliva glands.

Symptoms - Head - Mouth/Oral

(Very) dry mouth

In cases of Sjogren's syndrome, the body's immune system mistakenly attacks tear ducts and saliva glands, leading to chronically dry eyes and dry mouth (xerostomia).

(Minor/significant) mouth sores

Sjogren's Syndrome can result in painful mouth sores due to a dry mouth and/or Candida infection.


Symptoms - Head - Nose

Symptoms - Metabolic

Symptoms - Skeletal

Conditions that suggest Sjogren's Syndrome:


Gluten Sensitivity / Celiac Disease

Many Celiac Disease patients report they also have Sjogren's Syndrome, and vice versa.  Sjogren's Syndrome has been reported in up to 15% of patients with proven Celiac Disease.


Risk factors for Sjogren's Syndrome:



Cytomegalovirus (CMV) Infection

October, 2014: Australian researchers published their research findings in the journal Immunity that chronic cytomegalovirus (CMV) infection can lead to the development of Sjogren's syndrome, the second-most common autoimmune disease.  Some 50-80% of people in developed countries are infected with CMV, which is usually not a problem, but combined with a certain genetic background, chronic CMV can trigger autoimmunity.

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Sjogren's Syndrome suggests the following may be present:



Sjogren's Syndrome can lead to:


Chronic Thyroiditis

Antithyroid antibodies are created when antibodies migrate out of the salivary glands into the thyroid gland.  Antithyroid antibodies cause thyroiditis, a common problem in people with Sjögren's.


Sjogren's Syndrome could instead be:

Eyes / Ocular

Retinitis Pigmentosa

Sjogren's is an autoimmune disease sometimes misdiagnosed as retinitis pigmentosa. [Am J Ophthalmol, 1996 Dec, 122:6, pp.903-5 Abstract]

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Weak or unproven link: may be a sign or symptom of; may suggest; may increase risk of; is very occasionally misdiagnosed as
Weak or unproven link:
may be a sign or symptom of; may suggest; may increase risk of; is very occasionally misdiagnosed as
Strong or generally accepted link: is often a sign or symptom of; often suggests; often leads to
Strong or generally accepted link:
is often a sign or symptom of; often suggests; often leads to
Strong counter-indication: often contraindicates
Strong counter-indication:
often contraindicates
Definitely or absolutely counter-indicates: strongly contraindicates
Definitely or absolutely counter-indicates:
strongly contraindicates
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