REVIEW  HEPATITIS C INFECTION: A SYSTEMIC DISEASE 

Minerva Medica 2021 April;112(2):175-87

DOI: 10.23736/S0026-4806.20.07120-7

Copyright © 2020 EDIZIONI MINERVA MEDICA

lingua: Inglese

Hepatitis C virus-related cryoglobulinemic vasculitis

Cesare MAZZARO ¹ ✉, Endri MAURO ², Anna ERMACORA ³, Paolo DORETTO ⁴, Silvia FUMAGALLI ², Maurizio TONIZZO ³, Federica TOFFOLUTTI ⁵, Valter GATTEI ¹

¹ Unit of Clinical of Experimental Onco-Hematology, IRCCS Centro di Riferimento Oncologico (CRO), Aviano, Pordenone, Italy; ² Unit of Hematology, Department of Internal Medicine, Cà Foncello Hospital, Treviso, Italy; ³ Department of Internal Medicine, Pordenone General Hospital, Pordenone, Italy; ⁴ Unit of Laboratory, Pordenone General Hospital, Pordenone, Italy; ⁵ Unit of Cancer Epidemiology, IRCCS Centro di Riferimento Oncologico (CRO), Aviano, Pordenone, Italy

INTRODUCTION: Hepatitis C virus (HCV) infection affects about 170 million people worldwide. HCV is responsible for both hepatitis and extra-hepatic manifestations. Chronic infection has been shown to develop in about 70% of cases, and it can progress to cirrhosis or hepatocellular carcinoma. Ten percent of HCV patients may develop extra-hepatic manifestations, including mixed cryoglobulinemia (MC) and non-Hodgkin lymphomas (NHL). Cryoglobulinemic vasculitis (CV) varies, ranging from mild-moderate clinical symptoms (purpura on the legs, asthenia and arthralgias) and chronic hepatitis to severe symptoms (ulcers on the legs, peripheral neuropathy, glomerulonephritis, low-grade NHL to life threatening complications (rapid progressive glomerulonephritis, gastrointestinal vasculitis, acute hyper-viscosity).
EVIDENCE ACQUISITION: CV is associated with significant morbidity and mortality. Some studies have shown kidney involvement, cirrhosis, central nervous system involvement, and heart involvement as unfavorable prognostic factors. Many studies have demonstrated that, after antiviral therapy, CV can disappear along with HCV. After the introduction of the new direct antiviral agents (DAAs), the combination of pegylated interferon and ribavirin has been abandoned.
EVIDENCE SYNTHESIS: Several studies on new DAAs have reported remarkable 90% to 100% HCV eradication rates, regardless of genotype. Treatment with DAAs has comparable efficacy on viral eradication in CV patients but definite clinical improvements of vasculitis can be observed only in half the patients.
CONCLUSIONS: In patients with mild to moderate CV disease, DAAs therapy should be used as first line approach. In patients with severe vasculitis, DAAs therapy and a second-line treatment with RTX with or without aphaeresis are a required.

KEY WORDS: Cryoglobulinemia; Antiviral agents; Hepacivirus; Purpura; Arthralgias