• Cryoglobulinemia
  • Type I --> Monoclonal IgM and IgG, associated with Lymphoproliferative disorders
  • Type II --> Monoclonal IgM/Polyconal IgG
  • Type III --> Polyclonal IgM and IgG
  • Mixed Cryo (Type II and III) are associated with auto-immune disorders and infections (mainly HCV) as well
  • Pathophysiology
    • IgM and IgG binds antigens (ex. HCV) and fix complement resulting in immune deposits that set off an inflammatory response that can cause a number of clinical sequelae including leukocytoclastic vasculitis and glomerulonephritis.
  
  

  Posted 04/04/17 02:07:47 PM by Ying Liu

• Sjogren's Syndrome
  • Incidence of 5-10 per 100,000 people
  • Characterized by diminished lacrimal and salivary gland function with dryness of the eyes and mouth à Sicca Syndrome
  • Also associated with Extraglandular disease
    • Fatigue, myalgia, mild cognitive dysfunction
    • Cryoglobulinemia
      • Occurs in 16% of patients with Sjogrens
      • Usually Type II (Monoclonal/polyclonal) and mixed cryoglobulinemia
      • Monoclonal component is usually an IgM Kappa with RF activity
      • Can be associated with hypoclompementemia, leukocytoclastic vasculitis and HCV infection.
      • Potentially increased risk of developing B-cell lymphoma
    • Hypocomplementemia
    • Non-Hodgkin Lymphoma
    • ILD
  • Diagnosis:
    • Marker of dry eye or salivary hypofunction
    • SchirmerTest and ocular surface staining
    • Salivary Gland biopsy or Sialometry
    • Anti-Ro/SSA and Anti-La/SSB antibodies
  
  

  Posted 04/04/17 02:09:15 PM by Ying Liu

• Treatment of Sjogren's
  • Symptom Management
  • Prevention of dryness
  • Oral Hygiene/dental care
  • Saliva Substitutes and Sialagogues
  • Treatment of Mixed Cryoglobulinemia
  • Rituximab preferred (cyclophosphamide can also be considered)
  • Plasma Exchange
  • Symptomatic hyperviscosity
  • Life threatening disease
  • Rapidly progressive (crescentic) glomerulonephritis
  
  

  Posted 04/04/17 02:10:22 PM by Ying Liu

• For further reading...

  RCT of Rituxumab vs. Non-Rituxumab for Cryoglobulinemia
  
  

  Posted 04/04/17 02:13:08 PM by Ying Liu

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