6 min

Sjögren’s Syndrome- Causes, Symptoms, Diagnosis & Treatment

CONTENTS

What is Sjögren’s syndrome?

Sjögren’s (“SHOW-grins”) syndrome is an autoimmune disease that affects the entire body. It’s more common in women than men - with a female: male ratio of 9:1.

It mainly affects individuals between the ages of 40 and 60 (with the disease most frequently occurring in people around 50).

It affects glands that secrete fluid - such as your tear and salivary glands (leading to dry eyes and mouth). Other symptoms include fatigue and joint pain - but it can also affect your organs’ function (kidneys, intestines, blood vessels, lungs, liver, pancreas, or central nervous system). There is also an increased chance of developing lymphoma.

“Sjögren’s syndrome is more common in women than men - with a female: male ratio of 9:1”

SJÖGREN’S SYNDROME causes?

The exact cause of Sjögren’s syndrome is not known. Still, it is believed to be a combination of genetic, environmental, and hormonal factors:

  • Genetic factors: are believed to play a role in developing Sjögren’s syndrome. Studies have shown a higher incidence of the disease in families, suggesting that genetic mutations or variations may increase the risk of developing the condition.
  • Viruses: Epstein-Barr virus, cytomegalovirus, and hepatitis C virus have been linked to Sjögren’s syndrome. However, the relationship is not fully understood.
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SJÖGREN’S SYNDROME causes? (CONT.)

  • Environmental factors: such as exposure to certain viruses or bacteria, may trigger the immune system to attack the body’s glands.
  • Hormonal factors: also play a role in developing Sjögren’s syndrome. Women are more likely to develop the condition, suggesting that estrogen may play a role. The onset of Sjögren’s syndrome is also commonly associated with menopause when estrogen levels decrease.

Overall, Sjögren’s syndrome is a complex and multifactorial disease whose exact cause is not fully understood.

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“In some cases, lifestyle changes can help with specific symptoms - such as fatigue and gastrointestinal reflux. Over-the-counter products can help with dry eye and mouth symptoms.”

SJÖGREN’S SYNDROME - list of symptoms

  • Including concentration/memory loss, “brain fog”, dysautonomia, peripheral neuropathy, Raynaud’s syndrome and headaches.

  • Dry eyes, corneal ulcerations and infections. Preservative-free eye drops and sprays can help with these symptoms.

  • Including a dry nose, recurrent sinusitis and nosebleeds.

  • A dry mouth with mouth sores, dental decay, difficulty chewing, speech, and dentures. Swollen, painful parotid and salivary glands. Difficulty swallowing, heartburn, reflux and oesophagitis.

  • Recurrent bronchitis, interstitial lung disease, and pneumonia.

  • Joint and muscle pain, including arthritis.

  • Abnormal liver function tests, chronic active autoimmune hepatitis and primary biliary cholangitis.

  • Stomach upset, gastroparesis, irritable bowel, autoimmune gastrointestinal dysmotility.

  • Interstitial cystitis, interstitial nephritis including renal tubular acidosis, glomerulonephritis, and autoimmune pancreatitis. Vaginal dryness, vulvodynia, chronic prostatitis, autoimmune pancreatitis, fatigue, vasculitis, lymphoma, dry skin and skin sensitivity to UV light.

  • Treating Sjögren’s syndrome

    In some cases, lifestyle changes can help with specific symptoms - such as fatigue and gastrointestinal reflux. Over-the-counter products can help with dry eye and mouth symptoms.

    No single medicine has been conclusively proven to slow the progression of Sjögren’s syndrome or treat all aspects of the disease. Several different types of medications are used to manage symptoms:

    • Dry eye and dry mouth treatment.
    • NSAIDs (Non-Steroidal Anti-Inflammatory Drugs) and Corticosteroids to reduce inflammation.
    • DMARDs (Disease-Modifying Anti-Rheumatic Drugs) modify how the immune system functions.
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    Can flying worsen Dry Eyes & Sjögren's Syndrome?

    Air travel can intensify dry eye symptoms for people with Sjögren’s Syndrome. The low humidity levels in aeroplane cabins can cause more rapid evaporation of the tear film, leading to increased dryness and discomfort. Prolonged exposure to digital screens, air conditioning, and reduced blink rates during flights can further exacerbate these symptoms.

    To manage dry eye symptoms during a flight, follow our prevention tips (click the link below), such as using lubricating eye drops, staying hydrated, and taking breaks from screens. Also, speak to your eye specialist - they may suggest specific treatments, medications, or precautions for a more comfortable flying experience.

    Eye Care Guide To Healthy Flying

    “Most patients will, unfortunately, need prescription medication as their disease progresses - to help manage complications.”

    Diagnosing Sjögren’s syndrome

    There is no single test that confirms the diagnosis of Sjögren’s syndrome. Specialist doctors (normally rheumatologists) use several tests to diagnose Sjögren’s syndrome:

    Try Our 15-Second Blink Test

    Professor Fruttiger interview - Sjögren’s Syndrome & your eyes

  • Sjögren’s Syndrome is an autoimmune disease primarily affecting the body’s moisture-producing glands, including the tear and salivary glands. According to estimates, over 90% of people with Sjögren’s Syndrome suffer from dry eyes, making it one of the most prevalent and noticeable symptoms associated with the disorder. It is quite common for individuals with Sjögren’s Syndrome to experience dry eyes. In fact, dry eyes and mouth are this condition’s hallmark symptoms.

  • The primary eye-related symptom of Sjögren’s Syndrome is dry eyes, known as keratoconjunctivitis sicca. This condition is characterized by reduced tear production and can lead to various other symptoms, such as:

    • A gritty, sandy, or burning sensation in the eyes
    • Itching or redness
    • Blurred vision
    • Sensitivity to light
    • Eye fatigue
    • Excessive tearing, often as a reflex to eye irritation
    • Feeling like there’s a foreign object /something in your eyes
  • Tears play a crucial role in maintaining the health and comfort of the eyes, as they lubricate the ocular surface, remove debris, and help prevent infection. Sjögren’s Syndrome affects the eyes by attacking the lacrimal glands responsible for producing tears. When the immune system mistakenly targets these glands, they become inflamed, reducing tear production. Insufficient tear production disrupts the delicate balance of the eye, causing discomfort and increasing the risk of eye complications.

  • Several treatment options are available to manage dry eyes caused by Sjögren’s Syndrome. These may include:

    • Artificial tears: Over-the-counter lubricating, preservative-free eye drops & sprays can temporarily relieve dry eye symptoms by supplementing natural tear production.
    • Prescription eye drops: Medications such as cyclosporine (Restasis) or lifitegrast (Xiidra) can help increase tear production in some individuals.
    • Punctal plugs: Sometimes, a doctor may recommend inserting tiny silicone or collagen plugs into the tear ducts to reduce tear drainage and maintain eye moisture.
    • Warm compresses & eyelid hygiene: Regularly applying warm compresses and cleaning the eyelids can help alleviate dry eye symptoms and reduce inflammation.
    • Humidifiers: Using a humidifier to add moisture to the air may relieve dry eye discomfort, especially in dry environments.
    • Omega-3 fatty acid supplements: Studies suggest that omega-3 fatty acids may help improve dry eye symptoms in some people with Sjögren’s Syndrome.
  • While Sjögren’s Syndrome primarily causes discomfort and irritation in the eyes, it can potentially lead to more severe complications if left untreated. Chronic dry eyes can cause the eye’s surface to become more vulnerable to infections and abrasions. In severe cases, untreated, dry eye may result in corneal damage or even vision loss. Therefore, it is crucial to seek appropriate medical care and closely follow the treatment plan provided by your eye specialist.

  • Diagnosing Sjögren’s Syndrome-related eye problems usually involves a comprehensive eye exam by an ophthalmologist or optometrist in conjunction with a patient’s medical history, symptoms, and other diagnostic tests. Some of the tests include:

    • Schirmer’s test: This test measures tear production by placing a small strip of filter paper under the lower eyelid for a specific period. The amount of moisture absorbed by the paper indicates tear production levels.
    • Tear Break-Up Time (TBUT): This test assesses tear film stability. A dye is applied to the eye, and the time it takes for the tear film to break up and form dry spots is measured. A shorter tear breakup time indicates poor tear film stability, commonly seen in dry eye conditions. Try our 15-Second Blink Test and test your Tear Break-Up Time (TBUT).
    • Corneal and conjunctival staining: Fluorescein or other dyes are used to stain the eye’s surface, allowing the healthcare professional to visualize dry spots, damaged areas, or other abnormalities on the cornea and conjunctiva.
    • Meibomian gland assessment: The healthcare professional may examine the eyelids and the function of the meibomian glands, which produce the oily layer of the tear film. Dysfunction of these glands can contribute to dry eye symptoms.
    • Ocular surface assessment: A thorough examination of the eye’s surface, including the cornea, conjunctiva, and eyelids, can help identify signs of inflammation, damage, or other issues related to Sjögren’s Syndrome.
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    SJÖGREN’S SYNDROME - RESOURCES & REFERENCES

    1. Brito-Zerón, P., Baldini, C., Bootsma, H., Bowman, S. J., Jonsson, R., Mariette, X., ... & Ramos-Casals, M. (2016). Sjögren syndrome. Nature Reviews Disease Primers, 2, 16047. Retrieved from https://www.nature.com/articles/nrdp201647
    2. National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS). (2019). Sjögren's Syndrome. Retrieved from https://www.niams.nih.gov/health-topics/sjogrens-syndrome
    3. Fox, R. I. (2005). Sjögren's syndrome. The Lancet, 366(9482), 321-331. Retrieved from https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(05)66990-5/fulltext
    4. Mariette, X., & Criswell, L. A. (2018). Primary Sjögren's syndrome. New England Journal of Medicine, 378(10), 931-939. Retrieved from https://www.nejm.org/doi/full/10.1056/NEJMcp1702514
    5. Mavragani, C. P., & Moutsopoulos, H. M. (2014). Sjögren syndrome. Canadian Medical Association Journal, 186(15), E579-E586. Retrieved from https://www.cmaj.ca/content/186/15/E579
    6. The Johns Hopkins Jerome L. Greene Sjögren's Syndrome Center. (n.d.). Sjögren's Syndrome Information. Retrieved from https://www.hopkinssjogrens.org/disease-information/
    7. Sjögren's Syndrome Foundation. (n.d.). About Sjögren's. Retrieved from https://www.sjogrens.org/home/about-sjogrens
    8. Goules, A. V., Tzioufas, A. G., & Moutsopoulos, H. M. (2019). Sjögren's syndrome: a clinical review. Clinical Immunology, 198, 43-49.
    9. Fox, R. I., & Howell, F. V. (2011). Primary Sjögren's syndrome: clinical and immunopathological features. Seminars in Arthritis and Rheumatism, 21(3), 137-161.
    10. Nocturne, G., & Mariette, X. (2013). Sjögren Syndrome-associated lymphomas: an update on pathogenesis and management. British Journal of Haematology, 160(4), 458-465. Retrieved from https://onlinelibrary.wiley.com/doi/full/10.1111/bjh.12192
    11. Ramos-Casals, M., Solans, R., Rosas, J., Camps, M. T., Gil, A., Del Pino-Montes, J., ... & Font, J. (2008). Primary Sjögren syndrome in Spain: clinical and immunologic expression in 1010 patients. Medicine, 87(4), 210-219.