Sjögren syndrome

Sjögren syndrome 

Updated: 02/13/2021

© Jun Wang, MD, PhD

General features
  • Third most common rheumatic disorder, after rheumatoid arthritis and systemic lupus erythematosus
  • Typically presents with xerostomia, keratoconjunctivitis sicca (dry eye), rheumatoid arthritis, and hepergammaglobulinemia
  • More common in women, average onset between 40-60
  • May involve lymph nodes, lung, kidney, etc
  • Associated with Graves disease, Hashimoto thyroiditis, non-Hodgkin lymphoma, especially MALT lymphoma
Pathogenesis


Clinical features
  • Xerostomia (dry mouth)
  • Keratoconjunctivitis sicca (dry eyes)
  • Enlarged parotid glands
  • Rheumatoid arthritis
  • Hypergammaglobulinemia
Diagnostic criteria
  • Inclusion criteria: At least one symptom of ocular or oral dryness
  • Exclusion criteria
    • History of head and neck radiation treatment
    • Active hepatitis C infection
    • Acquired immunodeficiency syndrome
    • Sarcoidosis
    • Amyloidosis
    • Graft-verses-host disease
    • IgG4-related disease
Pathological features
Management
  • Relieving symptoms
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