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Understanding the Cryoglobulinemias

  • Alejandro Fuentes
  • Claudia Mardones
  • Paula I. BurgosEmail author
Vasculitis (L Espinoza, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Vasculitis

Abstract

Purpose of the Review

Cryoglobulins are immunoglobulins with the ability to precipitate at temperatures <37 °C. They are related to hematological disorders, infections [especially hepatitis C virus (HCV)], and autoimmune diseases. In this article, the state of the art on Cryoglobulinemic Vasculitis (CV), in a helpful and schematic way, with a special focus on HCV related Mixed Cryoglobulinemia treatment are reviewed.

Recent Findings

Direct – acting antivirals (DAA) against HCV have emerged as an important key in HCV treatment to related Cryoglobulinemic Vasculitis, and should be kept in mind as the initial treatment in non–severe manifestations. On the other hand, a recent consensus panel has published their recommendations for treatment in severe and life threatening manifestations of Mixed Cryoglobulinemias.

Summary

HCV-Cryoglobulinemic vasculitis is the most frequent form of CV. There are new treatment options in HCV-CV with DAA, with an important number of patients achieving complete response and sustained virologic response (SVR). In cases of severe forms of CV, treatment with Rituximab and PLEX are options. The lack of data on maintenance therapy could impulse future studies in this setting.

Keywords

HCV Mixed Cryoglobulinemia Type I Cryoglobulinemia gC1qR Direct-acting antivirals Rituximab 

Notes

Compliance with Ethical Standards

Conflict of Interest

No potential conflicts of interest relevant to this article were reported.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2019

Authors and Affiliations

  • Alejandro Fuentes
    • 1
  • Claudia Mardones
    • 1
  • Paula I. Burgos
    • 1
    Email author
  1. 1.Departamento de Inmunología clínica y ReumatologíaPontificia Universidad Católica de ChileSantiagoChile

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