• Clinical science

Sjogren syndrome

Summary

Sjögren syndrome is a chronic inflammatory autoimmune disease that occurs mainly in middle-aged women. The cause of primary Sjögren syndrome is unknown, whereas secondary Sjögren syndrome is associated with underlying autoimmune diseases (e.g., rheumatoid arthritis). As the immune system mainly attacks lacrimal and salivary glands, patients typically present with xerophthalmia (dry eyes) and xerostomia (dry mouth), the combination of which is also known as sicca syndrome. The disease may also involve the skin, joints, internal organs, and nervous system. It is diagnosed via the detection of autoantibodies (anti-SSA/Ro, ANA) and salivary gland biopsy. Management focuses on supportive measures and, in more severe cases, immunosuppressants.

Epidemiology

  • Sex: > (∼ 9:1)
  • Age of onset: : typically 40–60 years

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

References:[1][2]

Clinical features

Glandular symptoms

  • Inflammation of the salivary glands: decreased production of saliva → xerostomia (dry mouth)
    • Dysphagia
    • Increased formation of dental caries; and tendency to oral infections
    • Parotitis
  • Inflammation of the lacrimal glands (chronic dacryoadenitis): decreased secretion of tears → xerophthalmia (dry eyes)keratoconjunctivitis sicca
    • Redness, itching, burning of eyes
    • Sensation of sand or foreign body in the eyes
    • Blurred vision
  • Sicca syndrome: combination of dry mouth and dry eyes
  • Nasal dryness → chronic rhinitis, nosebleeds
  • Pharyngeal, tracheal, and bronchial dryness → persistent, dry cough
  • Vaginal dryness → dyspareunia (painful intercourse) and increased risk of infections

Extraglandular symptoms

  • General symptoms: : fatigue and arthralgias (∼ 70% of cases)
  • Skin manifestations: xerosis ; Raynaud's phenomenon (∼ 15–30% of cases)
  • Vasculitis (∼ 10% of cases)
  • Neurological and psychiatric manifestations
    • Depression
    • Variety of focal and/or diffuse findings (e.g., impaired gross motor control, paresis, seizures, peripheral neuropathy)
  • Gastrointestinal manifestations: e.g., dyspepsia, reflux esophagitis

The classical presentation is a middle-aged woman with dry eyes and a dry mouth, accompanied by rheumatoid arthritis or systemic lupus erythematosus!

References:[1][3][4][5][6][7]

Diagnostics

Sjögren syndrome is diagnosed based on the typical clinical features and is confirmed via detection of specific antibodies and a pathologically low tear production. Biopsy of the salivary gland is the most accurate test, but is not needed to establish the diagnosis.

Laboratory tests

Eye examinations

  • Schirmer's test: : shows decreased tear production
    • < 5 mm of moisture on the filter paper after 5 minutes are indicative of Sjögren syndrome
  • Slit lamp examination

Biopsy

References:[8][1][4][9]

Treatment

  • Treat the underlying disease in secondary Sjögren syndrome.
  • For dry mouth
    • Regular dental care
    • Adequate hydration and foods that stimulate salivary flow (e.g., dried fruit slices)
    • In moderate/severe disease: artificial saliva; , muscarinic agonists; , and immunosuppression (e.g., hydroxychloroquine)
  • For dry eyes
    • Avoid dry environments (e.g., shield eyes from wind, keep air humid)
    • Artificial tears
    • In moderate disease: topical immunosuppression (e.g., cyclosporine); , lubricants
  • Treatment of extraglandular disease depends on the specific manifestation: arthralgia may be managed with NSAIDs or hydroxychloroquine, whereas neurological manifestations, for instance, often require aggressive immunosuppression.

References:[10][11][12]

Complications

Increased risk of lymphomas (B-cell lymphoma, MALT lymphoma)!

References:[1][13]

We list the most important complications. The selection is not exhaustive.