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Sjögren Syndrome: Causes, Symptoms and Treatment

Sjögren syndrome is a chronic autoimmune disease that primarily attacks the glands producing tears and saliva, causing dry eyes and dry mouth.

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Things worth knowing about "Sjögren Syndrome"

Sjögren syndrome is a chronic autoimmune disease that primarily attacks the glands producing tears and saliva, causing dry eyes and dry mouth.

What is Sjögren Syndrome?

Sjögren syndrome is a chronic autoimmune disease in which the immune system mistakenly attacks the exocrine glands responsible for producing tears and saliva. This leads to the hallmark symptoms of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). The condition can occur on its own, known as primary Sjögren syndrome, or alongside other autoimmune diseases such as rheumatoid arthritis or systemic lupus erythematosus, referred to as secondary Sjögren syndrome. The disease predominantly affects women, with a female-to-male ratio of approximately 9:1.

Causes

The exact cause of Sjögren syndrome has not yet been fully established. It is believed to result from a combination of genetic predisposition, hormonal influences, and environmental triggers such as viral infections. The immune system incorrectly targets glandular tissue, causing chronic inflammation and damage.

Genetic factors: Certain HLA gene variants are associated with an increased risk of developing the disease.
Hormonal factors: The significantly higher prevalence in women, especially after menopause, suggests a role for sex hormones.
Infections: Viruses such as Epstein-Barr virus have been proposed as potential triggers.

Symptoms

Sjögren syndrome presents with a wide range of symptoms that extend well beyond the classic dryness complaints.

Classic Symptoms

Dry eyes: Burning, itching, a sensation of grit or sand in the eyes, and light sensitivity
Dry mouth: Difficulty swallowing and speaking, increased risk of dental decay
Enlarged salivary glands

Systemic Symptoms

Persistent fatigue
Joint and muscle pain
Dry skin and dry mucous membranes
Polyneuropathy (tingling or numbness in the hands and feet)
Kidney, lung, or liver involvement in severe cases

Diagnosis

Diagnosing Sjögren syndrome requires a combination of clinical evaluation, laboratory tests, and specialized procedures.

Schirmer test: Measures tear production using a small strip of filter paper placed under the eyelid.
Salivary flow test: Quantifies the amount of saliva produced over a defined period.
Blood tests: Detection of autoantibodies, particularly anti-SSA/Ro and anti-SSB/La, as well as markers of inflammation.
Lip biopsy: A tissue sample from the minor salivary glands of the inner lower lip to assess inflammatory changes.
Slit-lamp examination: An eye examination to evaluate the cornea and conjunctiva for signs of dryness-related damage.

Treatment

There is currently no cure for Sjögren syndrome. Treatment focuses on relieving symptoms, preventing complications, and improving quality of life.

Symptomatic Treatment

Artificial tears and eye drops to keep the eyes moist
Saliva substitutes and oral rinses to alleviate dry mouth
Regular dental check-ups to prevent tooth decay

Pharmacological Treatment

Pilocarpine or Cevimeline: Medications that stimulate saliva and tear production
Hydroxychloroquine: An antimalarial drug also used in autoimmune conditions to help with joint pain and fatigue
Nonsteroidal anti-inflammatory drugs (NSAIDs): For relief of joint and muscle pain
Corticosteroids and immunosuppressants: Used in cases of severe systemic involvement

Biologics

In severe cases, biological therapies such as rituximab (a monoclonal antibody targeting B-lymphocytes) may be considered, particularly when other treatments have not provided adequate relief.

Prognosis

Sjögren syndrome is typically a chronic but often stable condition. With appropriate management, most people can maintain a good quality of life. In rare cases, the disease is associated with an increased risk of developing lymphoma (a cancer of the lymphatic system), making regular medical follow-up essential.

References

Ramos-Casals M, Brito-Zeron P, Siso-Almirall A, Bosch X. Primary Sjogren syndrome. BMJ. 2012;344:e3821. DOI: 10.1136/bmj.e3821
Vivino FB. Sjogren's syndrome: Clinical aspects. Clinical Immunology. 2017;182:48-54. DOI: 10.1016/j.clim.2017.04.005
Mariette X, Criswell LA. Primary Sjogren's Syndrome. New England Journal of Medicine. 2018;378(10):931-939. DOI: 10.1056/NEJMcp1702514

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