Sjögren Syndrome

Sjögren Syndrome

 

Sjögren syndrome, a slowly progressive autoimmune disease, is characterized by lymphocytic infiltration of exocrine glands which results in dry eyes and dry mouth.

Epidemiology

  • Incidence – 2-4 million persons in U.S.
    • Second most common autoimmune disease
  • Age – peak 40-60 years
  • Gender – M:F; 1:9

Pathophysiology

  • Mononuclear infiltrate with loss of ductal cells and relative preservation of acinar cells in secretory glands
  • Leads to loss of secretory capacity of the gland

Clinical Presentation

  • Dry eye (xerophthalmia, keratoconjunctivitis sicca)
  • Dry mouth (xerostomia)
  • Enlargement of salivary glands
  • Arthritis
  • Raynaud phenomenon
  • Increased risk of lymphoma associated with Sjögren syndrome – mostly mucosally associated lymphoid tumors (MALT)
  • Often associated with other connective tissue diseases termed secondary Sjögren disease
    • Rheumatoid arthritis
    • Systemic lupus erythematous (SLE)
    • Primary biliary cirrhosis
    • Autoimmune thyroid disease

Diagnosis

  • Clinical features of connective tissue diseases overlap considerably. A systematic approach to laboratory diagnosis is recommended
  • Laboratory testing
    • ANA with followup ENA
    • SS antibodies – although not specific for Sjögren syndrome, antibodies are relatively sensitive  
      • Strong association between SSA antibodies and vasculitis in Sjögren syndrome
      • Several studies identify SSB antibodies as serological marker for SS-sicca complex
      • SSB antibodies detected in approximately 60% of SS-sicca complex patients
Distribution of Antibodies in Connective Tissue Disease Types
  Systemic Lupus Erythematosus (SLE) Sjögren Syndrome Mixed Connective Tissue Disease (MCTD) Progressive Systemic Sclerosis (PSS) Scleroderma
dsDNA abs 50-60% 20-30% 20-25% <5%  
Histone abs Idiopathic 18-53%
Drug-induced 80-95%		   <20% <20% <20%
RNP 20-30%   95-100% 15-25% 5-10%
Scl-70       25% 20-60%
SSA ANA positive patients
30-40%		 70-75%   5-10%  
SSB 15-25% 50-60%   5-10%  
Jo-1 abs Found in polymyositis, dermatomyositis, myositis associated with rheumatic disease



  • Histology
    •  May require labial gland biopsy

Differential Diagnosis

  • Hepatitis C
  • HIV
  • Sarcoidosis
  • Lymphoma

Treatment

  • Palliative

See Also