**Author details**

Aurore Fifi-Mah and Cheryl Barnabe

Department of Medicine, University of Calgary, Calgary, Canada

#### **References**


[5] J. C. Jennette, R. J. Falk, K. Andrassy, P. A. Bacon, J. Churg, W. L. Gross, E. C. Hagen, G. S. Hoffman, G. G. Hunder, C. G. Kallenberg, and et al., "Nomenclature of systemic vasculitides. Proposal of an international consensus conference," *Arthritis and Rheu‐ matism,* vol. 37, pp. 187-92, Feb 1994.

**10. Chapter summary**

208 Updates in the Diagnosis and Treatment of Vasculitis

refractory disease.

**Author details**

**References**

1990.

Aurore Fifi-Mah and Cheryl Barnabe

Department of Medicine, University of Calgary, Calgary, Canada

The treatment of AAV is directed at achieving disease control to prevent morbidity and mortality, while minimizing treatment toxicity. Corticosteroid use remains critical in rapidly achieving disease activity suppression, whereas cyclophosphamide and rituximab regimens should be reserved for induction of severe generalized disease, and plasma exchange for severe renal disease. In less severe cases of systemic disease methotrexate is suitable for remission induction. Maintenance of remission is achieved preferably with azathioprine or methotrexate, with leflunomide, mycophenolate mofetil and cyclophosphamide remaining as options. Finally, new discoveries and research will certify the role of alternative agents, such as monoclonal anti-tumor necrosis factor therapy, IVIg, DSG, ATG and CAMPATH-1, in

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**Chapter 9**

**Treatment of ANCA-Negative Small Vessel Vasculitis**

Small vessels refer to arterioles, capillaries and venules. According to an international consensus conference, small vessel vasculitides include, ANCA-associated vasculitis (granulomatosis with polyangitis [Wegener's], Churg-Strauss syndrome, microscopic pol‐ yangiitis), cryoglobulinaemic vasculitis, Henoch-Scholein purpura, and cutaneous leuko‐

Cryoglobulinaemia refers to circulating cryoglobulins. Cryoglobulins are immunoglobulins,

linaemia is classified in 3 types based on clonality and immunoglobulin class. In particular, type I consists of monoclonal IgM or IgG immunoglobulin, type II is a mixture of monoclo‐ nal IgM and polyclonal IgG, while type III is a mixture of polyclonal IgM and IgG. Type II and III are also called "mixed", since both contain a mixture of IgM and IgG immunoglobu‐ lins [3]. The IgM component of type II cryoglobulins has rheumatoid factor activity (can bind to the Fc portion of IgG). All 3 types of cryoglobulinaemia may or may not result from an underlying disorder. In the absence of an identifiable cause cryoglobulinaemia is charac‐

Circulating cryoglobulins induce damage through 2 mechanisms: type I monoclonal IgM cryoglobulins, due to the large size of IgM and their high concentration levels – usually as‐

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© 2013 Katsiari et al.; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

© 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

C and dissolve upon rewarming [2]. Cryoglobu‐

Christina G. Katsiari, Theodora Simopoulou and

Additional information is available at the end of the chapter

Lazaros I. Sakkas

**1. Introduction**

cytoclastic angiitis [1].

terized as "essential".

**2. ANCA-associated vasculitis**

which precipitate in temperatures below 37o

**2.1. Cryoglobulinaemic vasculitis**

http://dx.doi.org/10.5772/54272

