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

Epidemiology

Etiology

• 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

Clinical features

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

Treatment

Complications

• 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