Page 253 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 12

Cryoglobulinemic Vasculitis

Clodoveo Ferri?, M.D., Dilia Giuggioli, M.D. and
Marco Sebastiani, M.D.

Reumatologia, Universita di Modena e Reggio Emilia, Policlinico di Modena,
Via del Pozzo, Modena, Italy

Abstract

Cryoglobulinemic vasculitis, also termed mixed cryoglobulinemic syndrome, is a
rare systemic small vessel vasculitis due to the vascular deposition of immune-
complexes, mainly mixed IgG-IgM cryoglobulins. It is associated with hepatitis C virus
infection, immunological, and neoplastic diseases. Cryoglobulinemic vasculitis is
characterized by the classical triad of purpura, weakness and arthralgia, frequent multiple
organ involvement, and with infrequent late lymphatic and hepatic malignancies. The
etiopathogenesis of cryoglobulinemic vasculitis is not completely understood. However,
hepatitis C viral infection and associated lymphotropism, genetic and environmental
factors play important roles in cryoglobulin and immune-complex production that deposit
in blood vessels, and in B-lymphocyte expansion. The diagnosis is suggested by clinical
evidence of purpura, circulating mixed cryoglobulinemia and low C4 levels, and
pathologically evident leukocytoclastic vasculitis in skin biopsy lesions. The prognosis is
poor in patients with renal disease, liver failure, and malignancy. Treatment is directed
toward eradicating hepatitis C viral infection employing combination PEGylated-
interferon-alpha and ribavirin treatment, immunomodulatory and immunosuppressant
medications as warranted by the level of clinical severity.

Introduction

Cryoglobulinemic vasculitis (CV) is a small vessel vasculitis (SVV) due to the vascular
deposition of cryoprecipitable or non-cryoprecipitable immune-complexes (IC) and
complement [1]. Cryoglobulinemia and cryoimmunoglobulinemia are interchangeable terms

? Correspondence: Clodoveo Ferri M.D. E-mail address: [email protected].

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