Sjogren syndrome

Sjogren syndrome is a chronic inflammatory autoimmune disease that most commonly occurs in middle-aged women.

Epidemiology

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Etiology

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• Primary Sjogren syndrome: idiopathic (association with HLA-DR52)
• Secondary Sjogren syndrome: associated with another autoimmune disease, e.g., rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, polymyositis, or primary biliary cirrhosis

Pathophysiology

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Clinical features

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Sicca syndrome

• Ocular symptoms
  • Xerophthalmia: dry eyes due to decreased secretion of tears (daily, persistent)
  • Keratoconjunctivitis sicca
    • Conjunctival injection
    • Eye itching or burning sensation
    • Recurrent sensation of sand or a foreign body in the eyes
• Oral symptoms
  • Xerostomia: dry mouth due to decreased secretion of saliva (daily, persistent) which may lead to:
    • Dental caries and oral infections
    • Parotid gland enlargement, often bilateral
    • Tongue fissures

Diagnostics

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• Serology:
  • Anti-Ro (SSA) and Anti-La (SSB) antibodies: Highly specific, especially anti-La.
    • Sjogren syndrome → Anti-SSA and anti-SSB
  • Antinuclear Antibody (ANA): Positive in >95% of cases, making it a sensitive but non-specific test.
  • Rheumatoid Factor (RF): Often positive even without concomitant RA.
• Ophthalmologic Tests:
  • Schirmer test: Measures tear production by placing filter paper in the lower eyelid; <5 mm of wetting in 5 minutes is abnormal.
  • Rose bengal stain: Stains devitalized cornea and conjunctiva, revealing damage from dryness.
• Biopsy: Lip (minor salivary gland) biopsy is the most accurate test, showing focal lymphocytic infiltration.

Treatment

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Complications

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• Development of associated conditions
  • Autoimmune diseases, e.g., systemic lupus erythematosus, rheumatoid arthritis
  • B-cell lymphomas, e.g., MALT lymphoma
    • Prevalence ∼ 5%
    • Frequently manifests as unilateral, persistent parotid enlargement
    • Predictors of lymphoma include lymphadenopathy, palpable purpura, and cryoglobulinemia.
  • Renal tubular acidosis type 1
• Corneal scarring, ulcer, rupture, and infection
• Pregnancy: fetal loss, infant with neonatal lupus syndrome and associated complete heart block