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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Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4. Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas Multiple endocrine neoplasms 14. B
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