Abstract
Mixed cryoglobulinemia is the most prevalent extrahepatic manifestation of chronic HCV infection. It is usually a benign lymphoproliferative disorder which presents as vasculitis affecting different organs. Although life-threatening cryoglobulinemic vasculitis (CryoVas) is rare, it is sometimes the first and possibly lethal complication. Its treatment depends on the severity of vasculitis and can be challenging. High dose of corticosteroids, immunosuppressive agents and plasma exchange represent the first-line treatment, which should be followed by antiviral therapy. Rituximab is an effective and safe treatment option. However, the data about its use in life-threatening conditions are scarce. We report the case of a patient with severe, relapsing and life-threatening HCV-related CryoVas resistant to standard therapy who had had an initial beneficial response to rituximab added to plasma exchange that was later compromised by the development of sepsis. We also review the literature and discuss manifestations and therapy of life-threatening Cryovas with focus on rituximab use.
Highlights
Cryoglobulinemic vasculitis (CryoVas) is the most prevalent extrahepatic manifestation of chronic hepatitis C virus infection (HCV) which affects 150170 million people worldwide
Clinical picture of CryoVas is heterogeneous ranging from very mild symptoms to severe and life-threatening ones which may be the first manifestation of HCV infection [3]
Treatment of HCV-induced CryoVas depends on the severity of vascular lesions and it is challenging in patients with severe relapsing and life-threatening disease
Summary
Cryoglobulinemic vasculitis (CryoVas) is the most prevalent extrahepatic manifestation of chronic hepatitis C virus infection (HCV) which affects 150170 million people worldwide. Circulating mixed cryoglobulins are detectable in about 40% of HCVinfected individuals but overt CryoVas develops in only 5-10% of those [1] It is an immune-complex-mediated systemic vasculitis which most frequently affects small to medium sized blood vessels in the skin, joints and kidneys; any organ system can be involved [2]. Treatment of HCV-induced CryoVas depends on the severity of vascular lesions and it is challenging in patients with severe relapsing and life-threatening disease. We present our clinical experience with rituximab in the treatment of a patient with severe resistant, lifethreatening form of CryoVas, in whom initially beneficial response was followed by lethal septic condition. Despite intensive treatment with broad spectrum and antipseudomonal antibiotics, IVIg and other supportive therapy, the patient developed severe respiratory and renal failure that resulted in death from multiple organ dysfunction and septic shock
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