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Related Experiment Videos

Cryoglobulinaemia.

Dario Roccatello1, David Saadoun2,3, Manuel Ramos-Casals4,5,6

  • 1Center of Research of Immunopathology and Rare Diseases-CMID, Coordinating Center of the Network for Rare Diseases of Piedmont and Aosta Valley, and Nephrology and Dialysis Division (ERKnet member), Department of Clinical and Biological Sciences of the University of Turin, and San Giovanni Bosco Hospital, Turin, Italy. dario.roccatello@unito.it.

Nature Reviews. Disease Primers
|August 4, 2018
PubMed
Summary

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Mixed cryoglobulinaemia involves immunoglobulins precipitating in cold blood, often linked to Hepatitis C Virus (HCV). Treatment targets the underlying cause and vasculitis severity, including antiviral and immunosuppressive therapies.

Area of Science:

  • Immunology
  • Rheumatology
  • Infectious Diseases

Background:

  • Cryoglobulinaemia is characterized by immunoglobulins precipitating below 37°C.
  • Type I is monoclonal, while Types II and III are mixed cryoglobulinaemia (involving IgG and IgM).
  • Mixed cryoglobulinaemia is frequently associated with Hepatitis C Virus (HCV) infection, leading to B cell lymphoproliferation and autoantibody production.

Purpose of the Study:

  • This primer focuses on mixed cryoglobulinaemia.
  • To discuss the variable course and prognosis influenced by vasculitis-associated multiorgan damage.
  • To outline treatment strategies for cryoglobulinaemic vasculitis.

Main Methods:

  • Review of literature on mixed cryoglobulinaemia.
  • Analysis of associated disorders, particularly HCV.

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  • Discussion of clinical manifestations and treatment modalities.

    • Main Results:

    • Mixed cryoglobulinaemia can cause systemic vasculitis with diverse clinical manifestations, from purpura to severe organ damage.
    • Prognosis is significantly impacted by multiorgan involvement and the underlying associated disease.
    • Treatment requires a tailored approach based on the cause and severity, incorporating antiviral, immunosuppressive, and immunotherapy.

    Conclusions:

    • Mixed cryoglobulinaemia is a complex condition with significant morbidity and mortality.
    • Effective management necessitates addressing both the cryoglobulinaemia and its underlying etiology.
    • Therapeutic options include antivirals for HCV, immunosuppression, and targeted immunotherapies like anti-CD20 B cell depletion.