title: Cryofibrinogenemia: a marker of severity of cryoglobulinemic vasculitis.
abstract: BACKGROUND
  Cryofibrinogenemia 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.
  
  
  METHODS
  This 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).
  
  
  CONCLUSIONS
  Our results strongly suggest that presence of cryofibrinogenemia is associated with a more severe phenotype among patients with cryoglobulinemic vasculitis.
authors.affiliation: 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.
authors.forename: Martin
authors.initials: M
authors.lastname: Michaud
authors.affiliation: Toulouse University, Toulouse, France; Department of Internal Medicine, Toulouse University Hospital, France; INSERM UMR1027, Toulouse, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Moulis
authors.affiliation: Toulouse University, Toulouse, France; Immunology Laboratory, Toulouse University Hospital, France.
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Puissant
authors.affiliation: Department of Internal Medicine and Gerontology, Toulouse University Hospital, Toulouse, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Balardy
authors.affiliation: Department of Nephrology, Toulouse University Hospital, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Huart
authors.affiliation: Department of Internal Medicine, Joseph Ducuing Hospital, Toulouse, France.
authors.forename: Francis
authors.initials: F
authors.lastname: Gaches
authors.affiliation: Department of Internal Medicine, Cancer University Institute of Toulouse-Oncopole, Toulouse, France.
authors.forename: Pierre
authors.initials: P
authors.lastname: Cougoul
authors.affiliation: Toulouse University, Toulouse, France; Department of Internal Medicine, Toulouse University Hospital, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Arlet
authors.affiliation: Toulouse University, Toulouse, France; Immunology Laboratory, Toulouse University Hospital, France.
authors.forename: Antoine
authors.initials: A
authors.lastname: Blancher
authors.affiliation: Toulouse University, Toulouse, France; Department of Nephrology, Toulouse University Hospital, France.
authors.forename: Jacques
authors.initials: J
authors.lastname: Pourrat
authors.affiliation: Toulouse University, Toulouse, France; Department of Internal Medicine, Toulouse University Hospital, France; INSERM UMR1027, Toulouse, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Sailler
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D003449:Cryoglobulinemia / Q000097:blood* / Q000150:complications*; D003450:Cryoglobulins / Q000378:metabolism*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014657:Vasculitis / Q000097:blood* / Q000150:complications* / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D003450:Cryoglobulins; D007166:Immunosuppressive Agents
keywords: Cryofibrinogenemia; Cryoglobulinemia; Cryoglobulinemic vasculitis; Vasculitis