, David G. I. Scott2 and Chetan Mukhtyar2
Department of Rheumatology, Ipswich Hospital NHS Trust, Ipswich, UK
Department of Rheumatology, Norfolk and Norwich University Hospital, Norwich, UK
Cryoglobulinemic vasculitis is a rare medium/small vessel vasculitis that is often associated with Hepatitis C virus (HCV) infection.
The Chapel Hill Consensus Conference defined cryoglobulinemic vasculitis as vasculitis, with cryoglobulin immune deposits, affecting small vessels (predominately capillaries, venules, or arterioles), and associated with cryoglobulins in serum. Skin and glomeruli are often involved .
The epidemiology of cryoglobulinemic vasculitis has not been well described. It appears to be more common in areas with high rates of HCV infection especially Southern Europe compared with Northern Europe or the USA. The condition is more common in women than in men (3:1).
HCV was first identified in 1989. There is a strong association between HCV infection and essential mixed cryoglobulinemia, with 80–90 % of such patients being positive for anti-HCV antibodies. Circulating HCV-RNA has been identified in the peripheral blood of patients with cryoglobulinemia. HCV has been identified within cutaneous vasculitic lesions and has been selectively concentrated together with specific antibody in cryoprecipitates.
14.5 Clinical Features
In the early stages, the symptoms can be nonspecific and a high index of suspicion is required to achieve an early diagnosis. Meltzer’s triad (purpura, arthralgia, weakness) occurs in <40 % of patients .
A constitutional illness with fever, weight loss, myalgia, arthralgia commonly occurs.
The most typical feature is a purpuric rash. Less common features include urticaria, livedo, exanthem, acral necrosis, and leg ulcers. Raynaud’s phenomenon occurs in 20 %.
Polyneuropathy is present in 40–70 % (distal, symmetrical or asymmetrical, motor and/or sensory polyneuropathy, acute mononeuritis multiplex). Mononeuritis multiple is typically of acute onset. Peripheral neuropathy is typically of subacute gradual onset.
Renal involvement (nephrotic syndrome or nephritic urinary sediment due to mesangial proliferative glomerulonephritis occurs in <40 %.