Page 263 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Cryoglobulinemic Vasculitis 237

Figure 4. Therapeutic strategy of cryoglobulinemic vasculitis according to the activity and severity of
the disease. Therapeutic strategy of CV should be based on the activity/severity of clinical symptoms
and tailored for the single patient; in particular, in asymptomatic patients a careful monitoring is often
sufficient; other patients only sporadically show very mild manifestations, such as transient purpuric
lesions on the legs; in these cases monitoring may be sufficient, while an attempt at HCV eradication
may be considered. While in individuals with moderate-severe manifestations, mainly in the presence
of active chronic hepatitis, an attempt to eradicate the HCV infection should be carried out. Finally,
particularly severe, rapidly progressive complications must be treated with more aggressive treatments,
as in other systemic vasculitides; in individuals with particularly aggressive manifestations and/or
partial response to traditional therapies, sequential or combined treatment schedules can be employed.
Abbreviations: CV, cryoglobulinemic vasculitis; HCV, hepatitis C virus; CS, corticosteroids; LAC,
low-antigen-content; PEGylated-IFN?, PEGylated interferon-alpha; RIBA, ribavirin; CPX,
cyclophosphamide; RTX, rituximab; MCs, mixed cryoglobulinemic syndrome.

A LAC-diet improves the clearance of circulating IC by restoring the activity of the
reticuloendothelial system which becomes overloaded by circulating cryoglobulins [1, 4, 17].
It reduces the input of alimentary macromolecules crossing the mucosal barrier of the gut as
some foods such as dairy products and eggs present a potential antigenic activity and might
otherwise be pathogenic. Reduction of the alimentary input of macromolecules directed to
mononuclear phagocytic system improves its function in conditions characterized by
abnormal endogenous hyperproduction of IC responsible for organ damage such as MCs. An
LAC-diet and 2 to 4 mg per day of 6-methyl-prednisolone is sufficient to ameliorate mild
manifestations of CV such as sporadic purpura and arthralgia. The limitations of an LAC-diet
treatment include poor patient compliance which can be improved by tailoring the treatment
duration, for example 7 to 14 days of restricted diet followed by 14 to 28 days of free diet.
Those with mild-moderate symptoms such as palpable purpura who may be particularly

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