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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
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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
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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
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For further reading...
Posted 04/04/17 02:13:08 PM by Ying Liu
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