Sjögren's Syndrome

Definition | Aetiology | Pathophysiology | Risk Factors | Signs and Symptoms | Investigations | Management | References

Definition

Sjögren's Syndrome is a chronic autoimmune disorder characterised by the body's immune system attacking its moisture-producing glands, primarily affecting the salivary and lacrimal glands, leading to dry mouth (xerostomia) and dry eyes (keratoconjunctivitis sicca). It can occur as a primary condition or in association with other autoimmune diseases such as rheumatoid arthritis or lupus.

Aetiology

The exact cause of Sjögren's Syndrome is not fully understood, but it is believed to involve a combination of genetic, environmental, and hormonal factors. Key contributors include:

• Genetic predisposition, as certain HLA genes are associated with an increased risk
• Environmental triggers, such as viral infections, that may initiate or exacerbate the autoimmune response
• Hormonal influences, as the syndrome is more common in women, particularly those in middle age or postmenopausal
• Immune system dysregulation, where the body's immune cells attack the glands that produce saliva and tears

Pathophysiology

The pathophysiology of Sjögren's Syndrome involves:

• Lymphocytic infiltration of exocrine glands, particularly the salivary and lacrimal glands, leading to their destruction and reduced secretion
• Production of autoantibodies, including anti-Ro (SSA) and anti-La (SSB), which are markers of the disease
• Systemic inflammation, which can affect other organs such as the lungs, kidneys, liver, and nervous system, leading to a range of extra-glandular manifestations

Risk Factors

• Female gender, with a female-to-male ratio of approximately 9:1
• Age over 40, as the syndrome typically develops in middle-aged adults
• Family history of autoimmune diseases
• Presence of other autoimmune diseases, such as rheumatoid arthritis, systemic lupus erythematosus (SLE), or scleroderma
• Exposure to certain viruses, which may trigger the onset of the syndrome

Signs and Symptoms

The signs and symptoms of Sjögren's Syndrome include:

• Dry eyes (keratoconjunctivitis sicca), leading to irritation, a gritty sensation, and sensitivity to light
• Dry mouth (xerostomia), which may cause difficulty swallowing, speaking, and an increased risk of dental decay and oral infections
• Swelling and tenderness of the salivary glands, particularly the parotid glands
• Fatigue, which is a common and debilitating symptom
• Joint pain and stiffness, often resembling rheumatoid arthritis
• Skin dryness, rashes, or other skin abnormalities
• Extra-glandular manifestations, such as interstitial lung disease, peripheral neuropathy, or renal involvement

Investigations

Specific investigations to diagnose Sjögren's Syndrome include:

• Blood tests: Autoantibody testing for anti-Ro (SSA) and anti-La (SSB) antibodies, as well as other markers of autoimmune activity such as rheumatoid factor (RF) and antinuclear antibodies (ANA).
• Schirmer's test: To measure tear production and assess the severity of dry eyes.
• Salivary gland function tests: Sialometry to measure the flow rate of saliva and scintigraphy to evaluate salivary gland function.
• Labial gland biopsy: A biopsy of the minor salivary glands, often from the lower lip, may be performed to confirm the diagnosis by demonstrating lymphocytic infiltration.
• Imaging studies: Ultrasound or MRI of the salivary glands to assess glandular involvement and detect structural changes.

Management

Primary Care Management

• Symptomatic treatment: Use of artificial tears for dry eyes and saliva substitutes or sugar-free chewing gum to stimulate saliva production for dry mouth.
• Good oral hygiene: Regular dental check-ups, fluoride treatments, and the use of mouth rinses to reduce the risk of dental decay and oral infections.
• NSAIDs: Nonsteroidal anti-inflammatory drugs for managing joint pain and mild inflammation.
• Lifestyle modifications: Encouraging regular hydration, avoiding alcohol and caffeine (which can exacerbate dryness), and using a humidifier to alleviate dry air in the living environment.

Specialist Management

• Referral to rheumatology: For patients with significant systemic involvement or those requiring immunomodulatory therapy.
• Immunosuppressive therapy: Hydroxychloroquine may be prescribed for joint and skin symptoms, and systemic immunosuppressants (e.g., methotrexate, azathioprine) for severe systemic involvement.
• Topical treatments: Prescription eye drops such as cyclosporine (Restasis) may be used to reduce inflammation and increase tear production.
• Management of extra-glandular manifestations: Specialist input from pulmonology, nephrology, or neurology may be required for patients with lung, kidney, or nervous system involvement.
• Regular monitoring: Ongoing assessment of disease activity, organ involvement, and treatment side effects.

References

1. NHS (2024) Sjögren's Syndrome. Available at: https://www.nhs.uk/conditions/sjogrens-syndrome/ (Accessed: 24 June 2024).
2. National Institute for Health and Care Excellence (2024) Sjögren's Syndrome. Available at: https://cks.nice.org.uk/topics/sjogrens-syndrome/ (Accessed: 24 June 2024).
3. British Medical Journal (2024) Sjögren's Syndrome: Clinical Features, Diagnosis, and Management. Available at: https://www.bmj.com/content/350/bmj.h3020 (Accessed: 24 June 2024).
4. American College of Rheumatology (2024) Sjögren's Syndrome. Available at: https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Sjogrens-Syndrome (Accessed: 24 June 2024).

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