What is Sjogren Syndrome?
In the early 1900s, a Swedish doctor named Henrik Sjögren first noticed a group of women who had long-lasting arthritis along with dry eyes and dry mouth. Today, doctors who specialize in the treatment of arthritis and other joint conditions, called Rheumatologists, have a much better understanding of this syndrome, which was named after Sjögren. They know how this disorder affects the body system, and most importantly for the patients, they can provide advice about how to manage and live with it.
Primary Sjogren’s syndrome is a disorder where the body’s immune system incorrectly attacks its own tissues. It most commonly causes dryness symptoms, often affecting the eyes and mouth. This is because it leads to inflammation, and therefore damage, of the tear and saliva glands. Up to half of the people with this disorder may also experience effects in organs other than these glands, including the joints, skin, lungs, digestive system, nerves, and kidneys. This is described as extra-glandular involvement of the syndrome.
It’s also quite common for Sjogren’s syndrome to occur alongside other immune system disorders like rheumatoid arthritis and systemic lupus erythematosus. When this happens, it’s referred to as secondary Sjogren’s or Sjogren-overlap syndrome. The treatment methods usually focus on helping to replace the moisture in the affected glands and controlling the mistaken immune system attack, both in the specific area and throughout the body.
What Causes Sjogren Syndrome?
Sjogren syndrome is an illness of the immune system, which is when the body’s defenses mistakenly attack itself. Why people get this condition is still unknown, but there are clues. It seems like it is more likely to happen if you carry specific genes, which are parts of your DNA that determine many things about how your body works. Specifically, they’ve found that people with certain patterns in the HLA-DQA_DQB_ genes are at a higher risk. These patterns are seen in people around the world.
These genes are thought to cause Sjogren syndrome by affecting the immune system’s response to something in the environment. Think of it like this: because of these genes, your immune system may react too strongly to certain triggers in the environment, getting stuck in an attack mode and causing disease.
Studies from the lab and observations from patients suggest that viruses might be one of these environmental triggers. In particular, they have found hints that Epstein-Barr virus (EBV), a common virus that most people encounter at some point, might be involved in causing Sjogren syndrome.
Risk Factors and Frequency for Sjogren Syndrome
Sjogren syndrome is not an uncommon disease. It affects nearly half as many people as rheumatoid arthritis, representing about 0.5% to 1.0% of the general population.
- It’s estimated that between 400,000 to 3.1 million adults have Sjögren’s syndrome.
- People of all ages can get this condition, but it’s most usually seen in individuals between 45 and 55 years old.
- This disease co-occurs with rheumatoid arthritis or other connective tissue diseases, like lupus, in about half of the patients.
- Sjögren’s syndrome affects people around the globe and doesn’t seem to favor any specific race or geography. However, it is more common in women, with a female-to-male ratio of roughly 9 to 1.
- While this condition primarily presents in middle-aged individuals, it can occur in both the old and the young.
It’s important to note that due to a lack of appropriate referral tools to screen dry eye patients for Sjögren’s syndrome, there is a possibility that many cases of this disease remain undiagnosed.
Signs and Symptoms of Sjogren Syndrome
Patients suffering from Sjogren’s syndrome often describe a feeling like something is stuck in their eyes along with a dry mouth. During an eye examination, doctors may notice a lack of tears and redness in the eyes. However, a specialist eye doctor (ophthalmologist) needs to carry out a specific test, using a machine called a ‘slit-lamp’, to confirm the diagnosis. This test involves using a special dye to highlight any damage, and it’s key for spotting ‘dry eye’, the most common eye problem linked to Sjogren’s syndrome. To further check for dry eyes, doctors can also use the ‘Schirmer’ test.
The patient’s mouth might also appear dry, with a smooth tongue, and very thin layers of mucous. It’s also common for about half of all patients to have larger-than-normal salivary glands in the cheeks (parotid glands) and/or under the jaw (submandibular glands) at some time. Sometimes Sjogren’s syndrome can also cause symptoms in other parts of the body, like arthritis (joint inflammation), purpura (purple spots on the skin, caused by burst blood vessels), and/or signs of peripheral neuropathy (nerve damage).
Testing for Sjogren Syndrome
If your doctor suspects you might have Sjogren’s syndrome, a condition that primarily causes dry eyes and dry mouth, they will need to assess these symptoms to confirm the diagnosis. This might involve several tests such as a Schirmer test (which measures moisture in your eyes), an exam with a slit-lamp (a special microscope to examine your eyes), a salivary flow rate test (which checks how much saliva you can produce), and/or a nuclear scintigraphic evaluation (which uses a safe radioactive substance to create pictures of your salivary glands).
In addition, your doctor will likely take a blood test to look for certain autoimmune antibodies in your system. These are substances your body mistakenly produces as a reaction to healthy cells. The antibodies they’re looking for include ANA, RF, SS-A, and SS-B. Of these, SS-A is probably the most reliable for diagnosing Sjogren’s, but it can also be present in other autoimmune disorders and absent in some Sjogren’s cases.
The most specific test for diagnosing Sjogren’s involves a minor salivary gland biopsy. This is a small medical procedure where a tiny piece of tissue from your lower lip is removed and examined under a microscope. In patients with Sjogren’s, this will show a condition called focus lymphocytic sialadenitis (FLS), which is an inflammation of the salivary glands.
Treatment Options for Sjogren Syndrome
To relieve dryness symptoms (also known as sicca symptoms), we often recommend:
* Regular application of artificial tears during the day and gels at night for dry eyes. These usually don’t include preservatives.
* For severe dryness, small devices (punctal plugs) can block your tear ducts to keep your natural tears around longer. Sometimes, a small surgical procedure (cauterization) might be necessary to do so. It’s usually performed after trying plugs, as it might cause excessive tearing.
* Eye drops, like cyclosporine (also known as Restasis), can lessen inflammation in the glands around your eyes and help produce more tears.
* Serum tears, which are made from your own blood, can also help with dryness. Many eye centers can prepare these for you.
* Drinking water, chewing gum, or using artificial saliva might help with dry mouth.
Dryness can be eased with prescription medicines like pilocarpine (Salagen) or cevimuline (Evoxac) that help with saliva production. If you get a yeast infection, anti-fungal therapies might be needed. For dryness in the nose, humidifiers, and nasal saline (salt water) irrigation can be beneficial.
Medicines like proton-pump inhibitors and H2 blockers, which lessen the amount of acid in your stomach, may help with acid reflux, a condition where stomach acid gets into your esophagus.
Though these treatments help to lessen the dryness, some of it might persist. For more severe, whole-body (systemic) diseases, corticosteroids (a type of medication that reduces inflammation) can be used.
Various disease-modifying antirheumatic drugs (DMARDs) like methotrexate or azathioprine have also shown positive effects; they’re often used to reduce the need for steroids, although none have been officially approved for Sjogren’s syndrome, an immune system disorder causing dryness.
Hydroxychloroquine is recommended for treating inflammatory polyarthritis, a type of arthritis affecting multiple joints. Certain severe manifestations of the disease like inflammation of the arteries (vasculitis) might be helped by depleting the body of a type of immune cell (B cells). However, inhibitors of TNF-alpha, another type of medication that reduces inflammation, haven’t been shown to be effective for Sjogren’s syndrome.
What else can Sjogren Syndrome be?
There are numerous health conditions and habits that might prompt dry mouth. Some of these include:
- Sarcoidosis – a disease that causes inflammation and can impact numerous parts of the body
- Rosacea – a skin condition often causing facial redness
- Mumps – a viral infection affecting glands, particularly salivary ones
- Dehydration – not having enough fluid in your body
- Use of certain medications – specifically antidepressants and anticholinergics
- Mouth breathing, which may dry out the mouth
- Lymphoma – a type of cancer affecting the immune system
- Advanced age
- Parkinson’s disease – a disorder of the brain that leads to shaking and difficulty with walking, coordination, and movement
- Scleroderma – an autoimmune disease causing skin and connective tissues to thicken and harden
- Rheumatoid arthritis – a condition that causes painful inflammation in the joints
- AIDS (Acquired Immune Deficiency Syndrome) – the disease caused by HIV, which attacks the immune system
- Lupus – an autoimmune disease, where the body’s immune system attacks its own tissues and organs
