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- Martin Michaud, Guillaume Moulis, Bénédicte Puissant, Laurent Balardy, Antoine Huart, Francis Gaches, Pierre Cougoul, Philippe Arlet, Antoine Blancher, Jacques Pourrat, and Laurent Sailler.
- Department of Internal Medicine and Gerontology, Toulouse University Hospital, Toulouse, France; Institute National de la Recherche Medicale (INSERM) U858, Toulouse, France; Toulouse University, Toulouse, France. Electronic address: martin.michaud85@gmail.com.
- Am. J. Med. 2015 Aug 1; 128 (8): 916-21.
BackgroundCryofibrinogenemia is frequently associated with cryoglobulinemia. The aim of this study was to determine the characteristics associated with the presence of cryofibrinogenemia in patients with cryoglobulinemic vasculitis.MethodsThis was a single-center retrospective study that included patients with cryoglobulinemic vasculitis who were tested for cryofibrinogen at a tertiary referral center between January 1, 2011 and December 31, 2012. Twenty-nine patients fulfilled the CryoVas (cryoglobulinemic vasculitis) Survey criteria for cryoglobulinemic vasculitis. Eighteen patients had a detectable cryofibrinogen (CF-positive) and 11 had no detectable cryofibrinogen (CF-negative). Median cryoglobulin levels were 89 ± 129 mg/L in the CF-positive group and 68 ± 82 mg/L in the CF-negative group (P = .32). Median cryofibrinogen level was 70 ± 174 mg/L. Clinical manifestations were similar in both groups. Cancers and hematological disorders were more frequent among CF-positive patients (39% vs 0%, P = .026). Levels of alpha-1 and alpha-2 globulinemia were higher in the CF-positive group. Cryofibrinogenemia ≥ 100 mg/L was associated with cryoglobulinemic vasculitis (odds ratio [OR] 2.86; 95% confidence interval [CI], 1.06-7.73) in cryoglobulinemic patients. Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44).ConclusionsOur results strongly suggest that presence of cryofibrinogenemia is associated with a more severe phenotype among patients with cryoglobulinemic vasculitis.Copyright © 2015 Elsevier Inc. All rights reserved.
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