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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.

      Keywords

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      References

        • Korst D.R.
        • Kratochvil C.H.
        Cryofibrinogen in a case of lung neoplasm associated with thrombophlebitis migrans.
        Blood. 1955; 10: 945-953
        • Belizna C.
        • Loufrani L.
        • Subra J.F.
        • et al.
        A 5-year prospective follow-up study in essential cryofibrinogenemia patients.
        Autoimmun Rev. 2011; 10: 559-562
        • Michaud M.
        • Pourrat J.
        Cryofibrinogenemia.
        J Clin Rheumatol. 2013; 19: 142-148
        • Belizna C.C.
        • Tron F.
        • Joly P.
        • Godin M.
        • Hamidou M.
        • Levesque H.
        Outcome of essential cryofibrinogenaemia in a series of 61 patients.
        Rheumatology (Oxford). 2008; 47: 205-207
        • Saadoun D.
        • Elalamy I.
        • Ghillani-Dalbin P.
        • Sene D.
        • Delluc A.
        • Cacoub P.
        Cryofibrinogenemia: new insights into clinical and pathogenic features.
        Am J Med. 2009; 122: 1128-1135
        • Soyfoo M.S.
        • Goubella A.
        • Cogan E.
        • Wautrecht J.C.
        • Ocmant A.
        • Stordeur P.
        Clinical significance of cryofibrinogenemia: possible pathophysiological link with Raynaud's phenomenon.
        J Rheumatol. 2011; 39: 119-124
        • Delluc A.
        • Saadoun D.
        • Ghillani-Dalbin P.
        • Sene D.
        • Piette J.C.
        • Cacoub P.
        Cryofibrinogen in patients with hepatitis C virus infection.
        Am J Med. 2008; 121: 624-631
        • Jennette J.C.
        • Falk R.J.
        Small-vessel vasculitis.
        N Engl J Med. 1997; 337: 1512-1523
        • Terrier B.
        • Karras A.
        • Cluzel P.
        • et al.
        Presentation and prognosis of cardiac involvement in hepatitis C virus-related vasculitis.
        Am J Cardiol. 2013; 111: 265-272
        • Terrier B.
        • Krastinova E.
        • Marie I.
        • et al.
        Management of noninfectious mixed cryoglobulinemia vasculitis. Data from 242 cases included in the CryoVas survey.
        Blood. 2013; 119: 5996-6004
        • Blain H.
        • Cacoub P.
        • Musset L.
        • et al.
        Cryofibrinogenaemia: a study of 49 patients.
        Clin Exp Immunol. 2000; 120: 253-260
        • Musset L.
        • Diemert M.C.
        • Taibi F.
        • et al.
        Characterization of cryoglobulins by immunoblotting.
        Clin Chem. 1992; 38: 798-802
        • De Vita S.
        • Soldano F.
        • Isola M.
        • et al.
        Preliminary classification criteria for the cryoglobulinaemic vasculitis.
        Ann Rheum Dis. 2011; 70: 1183-1190
        • Nagy J.
        • Ambrus M.
        • Paal M.
        • Trinn C.
        • Burger T.
        Cryoglobulinaemia and cryofibrinogenaemia in IgA nephropathy: a follow-up study.
        Nephron. 1987; 46: 337-342
        • Sawada K.
        • Takahashi R.
        • Saniabadi A.R.
        • Ohdo M.
        • Shimoyama T.
        Elevated plasma cryofibrinogen in patients with active inflammatory bowel disease is morbigenous.
        World J Gastroenterol. 2006; 12: 1621-1625
        • Soyfoo M.S.
        • Watik A.
        • Stordeur P.
        • Gangji V.
        Cryofibrinogen levels are increased in non traumatic osteonecrosis: a new pathogenic clue to osteonecrosis?.
        Rheumatology (Oxford). 2013; 52: 1694-1700