Classification of Cryoglobulinemic Vasculitis

  • Salvatore De Vita
  • Luca Quartuccio


Classification criteria that have been developed with an accepted statistical methodology are presently lacking for cryoglobulinemic syndrome or vasculitis (CV). However, they are essential for research and epidemiologic studies, and in turn for clinical practice. Earlier CV classification criteria were elaborated by individual experts or a panel of experts, such as those proposed in 1995 by the GISC (Italian Study Group on Cryoglobulinemia). New, albeit still preliminary classification criteria for CV were recently developed by a co-operative international study drawing upon a standardized methodology based on a large number of real cases/controls (n = 925). These criteria were subsequently shown to have a high specificity (93.6%) and good sensitivity (88.5%) for CV. Formal validation involving experts from a larger number of countries is now needed.


Classification Criterion Systemic Vasculitis Work Plan Classification Study Cryoglobulinemic Vasculitis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


  1. 1.
    Meltzer M, Franklin EC (1966) Cryoglobulinemia – a study of twenty-nine patients. I. IgG and IgM cryoglobulins and factors affecting cryoprecipitability. Am J Med 40(6):828–836PubMedCrossRefGoogle Scholar
  2. 2.
    Gorevic PD, Frangione B (1991) Mixed cryoglobulinemia cross-reactive idiotypes: implications for the relationship of MC to rheumatic and lymphoproliferative diseases. Semin Hematol 28(2):79–94PubMedGoogle Scholar
  3. 3.
    De Vita S, De Re V, Gasparotto D et al (2000) Oligoclonal non-neoplastic B cell expansion is the key feature of type II mixed cryoglobulinemia: clinical and molecular findings do not support a bone marrow pathologic diagnosis of indolent B cell lymphoma. Arthritis Rheum 43(1):94–102PubMedCrossRefGoogle Scholar
  4. 4.
    Ferri C, Greco F, Longombardo G et al (1991) Antibodies to hepatitis C virus in patients with mixed cryoglobulinemia. Arthritis Rheum 34:1606–1610PubMedCrossRefGoogle Scholar
  5. 5.
    Agnello V, Chung RT, Kaplan LM (1992) A role for hepatitis C virus infection in type II cryoglobulinemia. N Engl J Med 327(21):1490–1495PubMedCrossRefGoogle Scholar
  6. 6.
    Johnson SR, Goek ON, Singh-Grewal D et al (2007) Classification criteria in rheumatic diseases: a review of methodologic properties. Arthritis Rheum 57:1119–1133PubMedCrossRefGoogle Scholar
  7. 7.
    Dougados M, Gossec L (2007) Classification criteria for rheumatic diseases: why and how? Arthritis Rheum 57: 1112–1115PubMedCrossRefGoogle Scholar
  8. 8.
    Mukhtyar C, Guillevin L, Cid MC et al (2009) European Vasculitis Study Group. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68:310–317PubMedCrossRefGoogle Scholar
  9. 9.
    Invernizzi F, Pietrogrande M, Sagramoso B (1995) Classification of the cryoglobulinemic syndrome. Clin Exp Rheumatol 13(Suppl 13):S123–S128PubMedGoogle Scholar
  10. 10.
    Vitali C, Bombardieri S, Moutsopoulos HM et al (1993) Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum 36:340–347PubMedCrossRefGoogle Scholar
  11. 11.
    Vitali C, Bombardieri S, Moutsopoulos HM et al (1996) Assessment of the European classification criteria for Sjögren’s syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren’s Syndrome. Ann Rheum Dis 55:116–121PubMedCrossRefGoogle Scholar
  12. 12.
    Vitali C, Bombardieri S, Jonsson R et al (2002) European Study Group on Classification Criteria for Sjögren’s Syndrome. Classification criteria for Sjögren’s syndrome: a revised version of the European criteria proposed by the American-European Consensus Group. Ann Rheum Dis 61:554–558PubMedCrossRefGoogle Scholar
  13. 13.
    Lamprecht P (2004) The cryoglobulinemic syndrome – report from the workshop on classification and on the 10th conference of the Italian Society for the Treatment of Cryoglobulinemia, Modena, 29 Sept, 2003. Z Rheumatol 63:235–238PubMedCrossRefGoogle Scholar
  14. 14.
    De Vita S, Soldano F, Isola M et al (2010) Preliminary classification criteria for the cryoglobulinemic syndrome. Ann Rheum Dis 69(Suppl 3):77Google Scholar
  15. 15.
    De Vita S, Soldano F, Isola M et al (2010) Preliminary classification criteria for the cryoglobulinemic syndrome. Arthritis Rheum 62(Suppl 10):S851–S852Google Scholar
  16. 16.
    De Vita S, Soldano F, Isola M et al (2011) Preliminary classification criteria for the cryoglobulinemic vasculitis. Ann Rheum Dis 70(7):1183–1190PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Italia 2012

Authors and Affiliations

  1. 1.Clinic of Rheumatology, Department of Medical and Biological SciencesAzienda Ospedaliero –Universitaria of UdineUdineItaly
  2. 2.Clinical Pathology and Clinic of RheumatologyAzienda Ospedaliero – Universitaria of UdineUdineItaly

Personalised recommendations