mixed cryoglobulinemia
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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
- peripheral neuropathy, mononeuritis
- glomerulonephritis
- cutaneous ulcers, skin infarctions
- 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
- treatment of HCV infection[3]
- immunosuppressive therapy
- suppression of B-cell clonal expansion & cryoglobulin production
- treatment of vasculitis[1]
- symptomatic
More general terms
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Additional terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 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: https://www.ncbi.nlm.nih.gov/pubmed/20399313
- ↑ 3.0 3.1 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: https://www.ncbi.nlm.nih.gov/pubmed/17566058