Sjögren's Syndrome

Sjögren's syndrome (SjS) is a long-term autoimmune disease primarily affecting the body's moisture-producing glands, particularly the lacrimal and salivary glands. It results in symptoms such as dry mouth, dry eyes, and can seriously affect other organ systems, including the lungs, kidneys, and nervous system.

Microscopic image of focal lymphoid infiltration in a minor salivary gland associated with Sjögren's syndrome

Symptoms

Primary Symptoms

• Dry mouth (xerostomia)
• Dry eyes (keratoconjunctivitis sicca)
• Fatigue
• Pain, particularly joint and muscle pain

Other symptoms include dry skin, vaginal dryness, chronic cough, numbness in the extremities, thyroid problems, blurred vision, constant eye discomfort, swollen parotid glands, dysphonia, difficulty swallowing, recurrent mouth infections, and debilitating fatigue.

Characteristic dryness at various locations, such as the tongue, face, and eyes

Complications

• Increased risk of lymphoma (15%)
• Kidney involvement leading to proteinuria and renal tubular acidosis
• Neonatal lupus erythematosus with congenital heart block in pregnant women with specific antibodies

Causes

While the exact cause is unknown, Sjögren's syndrome involves a combination of genetic predisposition and environmental triggers such as viral infections. It may occur independently (primary Sjögren's syndrome) or in association with other autoimmune diseases like rheumatoid arthritis, systemic lupus erythematosus, or systemic sclerosis.

Diagnosis

Diagnostic Methods

• Blood Tests: To detect antibodies indicative of Sjögren's syndrome, such as antinuclear antibodies (ANA) and rheumatoid factor (RF). Main antinuclear antibody patterns on immunofluorescence
• Rose Bengal Test: Uses dye to assess tear film and tear production.
• Schirmer Test: Measures tear production using a filter paper placed inside the lower eyelid.
• Saliva Flow Tests: Assess the production of saliva to diagnose dry mouth.
• Lip Biopsy: Examines tissue from the inner lip for lymphocytic infiltration and glandular damage.
• Ultrasound: Non-invasive imaging of salivary glands to reduce unnecessary biopsies.

Treatment

Symptomatic Management

• Dry Eyes: Artificial tears, cyclosporine (Restasis), punctal plugs, and goggles to retain moisture.
• Dry Mouth: Chewing sugar-free gum, sipping water, saliva substitutes, and medications like cevimeline (Evoxac) and pilocarpine (Salagen).
• Vaginal Dryness: Personal lubricants to alleviate irritation and pain.
• Musculoskeletal Pain: NSAIDs, corticosteroids, immunosuppressive drugs, and disease-modifying antirheumatic drugs (DMARDs) like hydroxychloroquine (Plaquenil) and methotrexate.

Systemic Treatments

• Fatigue and Joint Pain: Biologic immunosuppressant drugs such as rituximab and belimumab.
• Dental Care: Regular preventive treatments to manage xerostomia and prevent cavities.

Prognosis

Mortality and Quality of Life

Sjögren's syndrome is associated with a high burden of illness, significantly impacting quality of life and increasing the risk of disability. While life expectancy is generally normal, there is a notably high incidence of non-Hodgkin lymphoma among patients, with about 5% developing lymphoid malignancy.

Epidemiology

Sjögren's syndrome affects around 0.2% to 1.2% of the population, with a higher prevalence in women (90% of patients). The condition commonly begins in middle age but can occur at any age.

It is the third most common rheumatic autoimmune disorder, following rheumatoid arthritis and systemic lupus erythematosus.

History

Sjögren's syndrome was first described in 1933 by Henrik Sjögren, but earlier descriptions by Jan Mikulicz-Radecki in 1892 also documented similar symptoms. The disease was initially met with scepticism but gained recognition through Sjögren's extensive research and publications.

Carrie Ann Inaba, national awareness ambassador and spokesperson for the Sjögren's Syndrome Foundation

Research

Current research focuses on improving diagnostic tools, understanding genetic and environmental triggers, and developing effective treatments. The UK Primary Sjögren's Syndrome Registry supports clinical trials and genetic studies, aiming to advance knowledge and therapeutic options for this complex autoimmune disease.

Self-assessment MCQs (single best answer)

Which of the following is the primary symptom of Sjögren's syndrome?

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Which type of antibody is most commonly associated with Sjögren's syndrome?

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What is the hallmark diagnostic method for confirming Sjögren's syndrome?

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Which of the following is a common complication of Sjögren's syndrome?

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Which gender is more commonly affected by Sjögren's syndrome?

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Which treatment is commonly used to manage dry eyes in Sjögren's syndrome?

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What is the typical onset age range for Sjögren's syndrome?

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Which of the following is a non-invasive test that helps diagnose Sjögren's syndrome by measuring tear production?

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What is the relationship between Sjögren's syndrome and other autoimmune diseases?

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Which medication is used to stimulate saliva production in patients with Sjögren's syndrome?

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