Birmingham vasculitis activity score, disease extent index and complement factor C3c reflect disease activity best in hepatitis C virus-associated cryoglobulinemic vasculitis

Objective Clinical measures of vasculitis activity (Birmingham vasculitis activity sc ore = BVAS) and disease extent (Disease Extent Index = DEI), serological an d immunological parameters were evaluated for the monitoring of hepatitis C virus (HCV)-associated cryoglobulinemic vasculitis (CV), treated with eith er cyclophosphamide or interferon-alpha 2b depending on disease severity. Methods Serial serum samples of 15 patients with HCV-associated CV were analyzed, a nd BVAS, DEI, serological and immunological parameters were recorded at dia gnosis and during therapy. Eight patients were treated with interferon-alph a 2b and 7 patients with cyclophosphamide. Results A complete or partial response of the CV was seen in both treatment groups. BVAS, complement factor C3c, cryoglobulinemia, and rheumatoid factor signi ficantly decreased in both treatment groups during 6 months (p < 0.05). DEI decrease was significant in the cyclophosphamide group (p < 0.05), and the re was a trend in the interferon-alpha 2b group (p = 0.06). BVAS and DEI we re significantly positively correlated, and both parameters were significan tly negatively correlated with C3c levels in both treatment groups (interfe ron-alpha 2b/cyclophosphamide: r = -0.89, p = 0.001 versus r = -0.87, p < 0 .001, respectively) whereas other parameters were not, e.g. ESR and CRP. Conclusions Patients with different degrees of disease severity, treated with either cy clophosphamide or interferon-alpha 2b depending on their disease activity, achieved remission of their CV. BVAS, DEI and C3c were especially useful in the follow-up of HCV-associated CV. C3c correlated with BVAS and DEI durin g therapy and provided additional information about vasculitis activity tha t was not reflected by other serological or immunological parameters, e.g. ESR or CRP.