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著者: Benjamin Terrier, Isabelle Marie, Adeline Lacraz, Pauline Belenotti, Fabrice Bonnet, Laurent Chiche, Bruno Graffin, Arnaud Hot, Jean-Emmanuel Kahn, Catherine Michel, Thomas Quemeneur, Luc de Saint-Martin, Olivier Hermine, Jean-Marc Léger, Xavier Mariette, Patricia Senet, Emmanuelle Plaisier, Patrice Cacoub
雑誌名: J Autoimmun. 2015 Dec;65:74-81. doi: 10.1016/j.jaut.2015.08.008. Epub 2015 Aug 29.
Abstract/Text
In patients with infectious cryoglobulinemia vasculitis (CryoVas) in the absence of hepatitis C virus infection, data on presentation, therapeutic management and outcome are lacking. We conducted a nationwide survey that included patients with HCV-negative CryoVas. We describe here the presentation, therapeutic management and outcome of 18 patients with non-HCV infectious CryoVas and 27 additional patients identified form a systematic review of the literature. We included 18 patients, mean age 57.9±13.5 years. Infectious causes were viral infections in 8 patients [hepatitis B virus (HBV) in 4, and cytomegalovirus, Epstein Barr virus, parvovirus B19 and human immunodeficiency virus in one case each], pyogenic bacterial infection in 6 patients, parasitic infection in 2 patients, and leprosy and candidiasis in one case each. Baseline manifestations were purpura (78%), glomerulonephritis (28%), arthralgia (28%), peripheral neuropathy (22%), skin necrosis (22%), cutaneous ulcers (17%), and myalgia (11%). Cryoglobulinemia was type II in 2/3 of cases. Most cases received specific anti-infectious therapy as first-line therapy, sometimes associated with corticosteroids, achieving sustained remission in the majority of cases. Refractory or relapsing patients, frequently related to HBV infection, showed a complete remission after rituximab in addition to antiviral therapy. In contrast, corticosteroids and/or immunosuppressive agents used in the absence of anti-infectious agents were frequently associated with refractory CryoVas. Viral and pyogenic bacterial infections represent the main causes of non-HCV infectious CryoVas. Antimicrobial therapy is commonly associated with sustained remission. Immunosuppressive agents should be considered only as a second-line option in patients with refractory vasculitis.
Copyright © 2015 Elsevier Ltd. All rights reserved.
PMID 26320984 J Autoimmun. 2015 Dec;65:74-81. doi: 10.1016/j.jaut.2015.08.008. Epub 2015 Aug 29.
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