What is Sjogren's Syndrome?

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First described in the early 1900s, Sjogren’s syndrome is considered to be the second most prevalence multisystem autoimmune condition following rheumatoid arthritis.

What is Sjogren’s Syndrome

Thought to develop between the ages of 40 to 60, Sjogren’s syndrome is a condition which typically affects the areas of the body responsible for the synthesis of fluids such as saliva and tears. The syndrome is a chronic autoimmune-mediated systemic inflammatory disorder, which as, a long-term condition, can severely impair the ability to manage daily life. The main symptoms experienced by those with the condition include dry eyes and a dry mouth (known as sicca symptoms).

There are two main types of Sjogren’s syndrome. Primary Sjogren’s syndrome which develops in individuals with no other underlying cause, and secondary Sjogren’s syndrome. The latter can be caused as a result of an associated rheumatologic disease such as rheumatoid arthritis and systemic lupus erythematosus.

Symptoms of Sjogren’s Syndrome

The clinical symptomology of Sjogren’s syndrome can be categorized by those linked to exocrine dysfunction (glandular) and those that typically affect the bodily organs other than those exocrine glands (systemic). Therefore, there is a range of symptoms that may be experienced in those with Sjogren’s syndrome, including:

  • Swollen salivary glands
  • Ocular dryness
  • Dry mouth
  • Upper respiratory tract dryness
  • Vaginal dryness
  • Dry skin
  • Fatigue
  • Joint or muscle pain

Complications of Sjogren’s Syndrome

The symptoms associated with Sjogren’s syndrome can cause a series of knock-on complications. For example, dryness of the eyes may create a burning sensation or make them feel gritty. Whereas, a dry mouth may result in difficulty eating and swallowing food. Furthermore, research has found that in 70% of those with the condition, easy fatigability is one of the most commonly occurring symptoms. There is often the increased need for rest and increased functional disability reported in such patients. Other common complications are outlined below.

Lung Issues

Lung complications can develop in those with Sjogren’s syndrome. Specifically, those with the condition may be more at risk of developing lung infections and bronchiectasis alongside scarring of the lung tissue. Due to this, those with Sjogren’s syndrome are often recommended not to smoke or given help to stop smoking to avoid worsening the issue. It’s also quite common for those with the condition to have a cough. However, in instances where shortness of breath, wheezing or a having a cough for a prolonged period is experienced, then it is recommended that further medical help is sought.

Pregnancy Complications

Although the majority of women with Sjogren’s syndrome can get pregnant and carry healthy babies, women are advised to speak to their doctor when planning to conceive due to potential complications. The main complications are heart issues in the baby and a rash that is present on the baby that lasts a few weeks postnatally.

Cancer

Research has found that those with Sjogren’s syndrome are approximately five times more likely to develop non-Hodgkin lymphoma - a type of cancer - than those without Sjogren’s syndrome.

Eye Issues

If dry eyes persist and are not treated, then there is a risk that the outermost layer of the eyes could be damaged. If it is not treated at all, then permanent issues with vision may occur.

In addition to these, there is a range of other complications that those with Sjogren’s syndrome may face. These include irritable bowel syndrome, underactive thyroid glands and kidney problems such as kidney stones and inflammation.

How is Sjogren’s Syndrome Diagnosed?

As the symptomology of Sjogren’s syndrome overlaps with several other health conditions, the syndrome can be quite challenging to diagnose. Therefore, referral to a specialist and further exploratory testing may be required.

An ophthalmologist may look at the eye to examine the layer of tears present. If the layer appears to be inconsistent and patchy, then this may be indicative of Sjogren’s syndrome.

Blood testing may be carried out to identify the presence of a set of antibodies that are characteristic of Sjogren’s syndrome. However, some people that have the syndrome do not have the antibodies. Therefore, patients may still have the syndrome despite a negative blood test result.

A lip biopsy may also be carried out to investigate whether groups of white blood cells reside in cells where saliva is synthesized.

How is Sjogren’s Syndrome Treated?

The majority of treatment methods for Sjogren’s syndromes aim to address the sicca symptoms experienced, alongside the fatigue.

Strategies to reduce the severity of dry eye symptoms include:

  • Using a humidifier to increase moisture in the air
  • Shielding the eyes from wind by wearing wraparound glasses
  • Reducing the time spent looking at devices and reading

Over-the-counter eye drops or gels may be taken for milder cases in order to ease dry eyes. If the eye is irritated, then steroid-containing drops may be prescribed. Anti-inflammatory eye drops may be prescribed, or in more severe cases, patients may undergo surgery to have a punctual plug inserted which blocks drainage from tear ducts and reduces the occurrence of dry, itchy eyes.

To address, a dry mouth, a cholinergic agonist may be taken by the patient to increase salvia production. Non-steroid anti-inflammatory drugs can also be used to manage arthritis-like symptoms associated with the syndrome.

Prevention and avoidance strategies can be put in place for patients to follow to help mitigate some of the symptoms. For example, stopping smoking and using fluoride toothpaste to reduce tooth decay, as well as promoting the avoidance of activities that exacerbate fatigue or recommending fitness to increase aerobic endurance.

Sources

  • NHS (2017). Sjogren’s Syndrome: Overview. https://www.nhs.uk/conditions/sjogrens-syndrome/
  • Mavragani C. P., & Moutsopoulos H. M. (2014). Sjogren syndrome. Canadian Medical Association Journal. DOI: 10.1503/cmaj.122037
  • Flament, T., Bigot A., Chaigne B., et al. (2016). Pulmonary manifestations of Sjogren’s syndrome. European Respiratory Review. DOI: 10.1183/16000617.0011-2016
  • Stefanski, A-L., Tomiak, C., Pleyer, U., et al. (2017). The Diagnosis and Treatment of Sjogren’s Syndrome. Deutsches Arzteblatt. DOI: 10.3238/arztebl.2017.0354

Further Reading

Last Updated: Oct 10, 2019

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