Page 257 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Cryoglobulinemic Vasculitis 231

Table 1. Cryoglobulinemic vasculitis: Demographic, Clinicoserological and Virological
Findings at a Single Institution*

Total Number of Patients 270
Mean age at disease-onset (± SD), years (range) 55 (±14) (29-74)
Female/Male ratio 3:1
Disease duration, mean ± SD years (range) 14 (±11) (2-42)
Purpura 98%
Weakness 98%
Arthralgia 93%
Non-erosive arthritis 9%
Raynauds phenomenon 32%
Sicca syndrome 51%
Peripheral neuropathy 81%
Renal involvement 32%
Liver involvement 73%
B-cell non-Hodgkin lymphoma 12%
Hepatocellular carcinoma 3%

Mean cryocrit% (± SD) 4.1 (±14)
Type II/type III mixed cryoglobulins 2/1
Mean C3 (± SD) in mg/dl (normal 60-130) 91 (±35)
Mean C4 (± SD) in mg/dl (normal 20-55) 10 (±14)
Antinuclear antibodies 31%
Antimitochondrial antibodies 9%
Anti-smooth muscle antibodies 15%
Anti-extractable nuclear antigen antibodies 9%
Anti-HCV RNA 92%
Anti-HBV antibodies 30%
HBsAg 1%

*Evaluated at the end of the patient followup period.

There are different presenting clinical-serological patterns that vary from mild arthralgia
and sporadic purpura in those with MC alone, to more severe cutaneous and systemic
involvement of classical MCs [1-4]. In all such patients, the disease combines MC with RF
activity, low C4 levels, and the clinical-pathological features of orthostatic purpura due to
leukocytoclastic vasculitis (LCV) with variable multiorgan involvement. Patients chronically
infected with HCV demonstrate asymptomatic serum MC that precedes the clinical onset of
disease by years to decades [1, 4].

Conversely, some patients show typical cryoglobulinemic manifestations without serum
cryoglobulins. The absence of this serological hallmark is not surprising since cryoglobulins
represent only a portion of IC that cryoprecipitate, which may be transient and vary both
among patients and during follow-up in individual patients [1, 4]. Most experts agree that
repeated cryoglobulin determinations are necessary for a definite diagnosis in such patients.
Cutaneous manifestations are the commonest in CV, with orthostatic purpura, the
pathognomonic feature, varying largely in dimension and diffusion to sporadic isolated

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