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mixed cryoglobulinemia
Etiology:
- > 90% associated with hepatitis C (2/3) [1]
- connective tissue disorders
- lymphoproliferative disorders
Clinical manifestations:
- triad of
- palpable purpura
- arthralgia (without arthritis)
- muscle weakness, myalgias
- may be associated with
b) peripheral neuropathy, mononeuritis
c) glomerulonephritis
c) cutaneous ulcers, skin infarctions
d) widespread vasculitis [1]
Laboratory:
- cryoglobulins in serum/plasma
- trace amounts of cryoglobulins may be found in normal individuals
- complement C4 is low*
- complement C3 is generally normal* [1]
- rheumatoid factor is generally positive
- urinanalysis - RBC, WBC, RBC casts
- serum creatinine may be elevated
- serum protein electrophoresis
- polyclonal IgG with monoclonal IgM (or polyclonal IgM) [1]
- M spike is the rheumatoid factor [1]
* distinguishing from lupus erythematosus
Management:
1) treatment of HCV infection [3]
2) immunosuppressive therapy
- suppression of B-cell clonal expansion & cryoglobulin production
- treatment of vasculitis [1]
3) symptomatic
Related
cryoglobulin
Specific
type 2 (mixed) cryoglobulinemia
type 3 (mixed) cryoglobulinemia
General
cryoglobulinemia (cryoglobulinemic vasculitis)
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 17, 18, 19.
American College of Physicians, Philadelphia 2009, 2015, 2018, 2022.
- Iannuzzella F, Vaglio A, Garini G.
Management of hepatitis C virus-related mixed cryoglobulinemia.
Am J Med. 2010 May;123(5):400-8
PMID: 20399313
- Saadoun D, Landau DA, Calabrese LH, Cacoub PP.
Hepatitis C-associated mixed cryoglobulinaemia: a crossroad
between autoimmunity and lymphoproliferation.
Rheumatology (Oxford). 2007 Aug;46(8):1234-42. Epub 2007 Jun 12.
PMID: 17566058