Cryoglobulinemic vasculitis: having giant steps; but there are still unanswered questions
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Cryoglobulinemic vasculitis: having giant steps; but there are still unanswered questions Omer Karadag1,2 · Emine Duran1,2 Received: 20 June 2020 / Accepted: 20 August 2020 © Società Italiana di Medicina Interna (SIMI) 2020
Cryoglobulinemic vasculitis (CryoVas) is a systemic immune-complex vasculitis which can be a life-threatening condition. In their article recently published by Internal and Emergency Medicine, Vacchi C et al. reported the safety and effectiveness of biosimilar of Rituximab CT-P10 in the treatment of CryoVas [1]. For a better understanding of this article, an overarching look to the historical picture of CryoVas and details of the usage of biosimilars will be helpful. Cryoprecipitate was first described in a multiple myeloma patient in 1933 [2]. In the paper of 1950′s, etiologies among 121 patients with cryoglobulinemia, were multiple myeloma (n = 14), disseminated lupus (n = 6), alcoholics (n = 23), chronic hepatitis (n = 1) and miscellaneous (n = 45) [3]. Enormous improvements had been observed regarding etiopathogenesis and treatment modalities of this concept (Fig. 1) [4]. In a recent French cohort, 56.1% of patients with mixed cryoglobulinemia (MC) were HCV-related [5]. Others were autoimmune diseases, essential, hematological/neoplastic, and infections in decreasing order. Circulating cryoglobulins may be detected in approximately 40% of HCV-infected patients, asymptomatically. CryoVas occurs in a minority of cases ( 95%) SVR. These agents lead to significant decrease in the incidence of MC and CryoVas. Low-dose corticosteroids may be used for constitutional and musculoskeletal features in CryoVas [6, 7]. Antiviral treatment solely was not enough to treat severe patients (ie, glomerulonephritis, mononeuritis multiplex, extensive cutaneous ulcers). The conventional immunosuppressives along with corticosteroids have been used without evidence from randomized controlled trials [7]. However, the outcomes with these modalities were not satisfied. Biologic agents are milestones of inflammatory rheumatic diseases including vasculitis. The efficacy of rituximab (RTX) for treatment of severe cryoglobulinemic vasculitis has been demonstrated in randomized controlled trials [8]. Currently, RTX is recommended to control disease with or without plasmapheresis. It is usually required before initiation of antiviral therapy [6, 7]. Moreover, efficacy of lowdose RTX (250 mg/m2 two times one week apart) in HCVrelated CryoVas was reported [8]. Comparison of these two dosing regimens is necessary to define optimum dosage of RTX. Other area which had scarce data is the place of RTX in non-HCV-related CryoVas. The economic impact of biologic agents is expected to be huge day by day. A RTX-biosimilar, CT-P10 was approved in Europe with significant budget savings. With a changing
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Internal and Emergency Medicine
Descrip on of cryoprecipita on in a mul ple myelom pa ent, 1933
1930
Defini on of triad: purpura, weakness and arthralgia; Introduc on of term of essen al
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