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Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey
  1. Benjamin Terrier1,
  2. Fabrice Carrat2,
  3. Evguenia Krastinova2,
  4. Isabelle Marie3,
  5. David Launay4,
  6. Adeline Lacraz5,
  7. Pauline Belenotti6,
  8. Luc de Saint Martin7,
  9. Thomas Quemeneur8,
  10. Antoine Huart9,
  11. Fabrice Bonnet10,
  12. Guillaume Le Guenno11,
  13. Jean-Emmanuel Kahn12,
  14. Olivier Hinschberger13,
  15. Patricia Rullier14,
  16. Aurelie Hummel15,
  17. Elisabeth Diot16,
  18. Christian Pagnoux17,
  19. Estibaliz Lzaro10,
  20. Frank Bridoux18,
  21. Thierry Zenone19,
  22. Olivier Hermine20,
  23. Jean-Marc Leger21,
  24. Xavier Mariette22,
  25. Patricia Senet23,
  26. Emmanuelle Plaisier15,
  27. Patrice Cacoub24
    1. 1Department of Internal Medicine, Cochin Hospital, Paris, France
    2. 2Biostatistics Department, Saint Antoine, Paris, France
    3. 3Department of Internal Medicine, CHU, Rouen, France
    4. 4Department of Internal Medicine, CHU, Lille, France
    5. 5Department of Nephrology, CHU, Bordeaux, France
    6. 6Department of Internal Medicine, CHU, Marseille, France
    7. 7Department of Internal Medicine, CHU, Brest, France
    8. 8Department of Internal Medicine, CH, Valenciennes, France
    9. 9Department of Nephrology, CHU, Toulouse, France
    10. 10Department of Internal Medicine, CHU, Bordeaux, France
    11. 11Department of Internal Medicine, CHU, Clermont Ferrand, France
    12. 12Department of Internal Medicine, CH, Suresnes, France
    13. 13Department of Internal Medicine, CH, Mulhouse, France
    14. 14Department of Internal Medicine, CHU, Montpellier, France
    15. 15Department of Nephrology, CHU, Paris, France
    16. 16Department of Internal Medicine, CHU, Tours, France
    17. 17Department of Internal Medicine, CHU, Paris, France
    18. 18Department of Nephrology, CHU, Poitiers, France
    19. 19Department of Internal Medicine, CH, Valence, France
    20. 20Department of Hematology, CHU, Paris, France
    21. 21Department of Neurology, CHU, Paris, France
    22. 22Department of Rheumatology, CHU, Kremlin Bicetre, France
    23. 23Department of Dermatology, CHU, Paris, France
    24. 24Department of Internal Medicine, CHU, Paris, Tennessee, USA
    1. Correspondence to Patrice Cacoub, Department of Internal Medicine, Pitié-Salpétrière Hospital, 47-83 boulevard de l'Hôpital, Paris 75013, France; patrice.cacoub{at}psl.aphp.fr Benjamin Terrier, Department of Internal Medicine, Cochin Hospital, 17 rue du Faubourg Saint-Jacques, Paris 75014, France; benjamin.terrier{at}cch.aphp.fr

    Abstract

    Background Data on the prognosis of non-infectious mixed cryoglobulinaemia vasculitis (CryoVas) in the era of hepatitis C virus screening are lacking.

    Methods The French multicentre and retrospective CryoVas survey included 242 patients with non-infectious mixed CryoVas. Causes of death and prognostic factors of survival were assessed and a prognostic score was determined to predict survival at 5 years.

    Results After a median follow-up of 35 months, 42 patients (17%) died. Causes of death were mainly serious infections (50%) and vasculitis flare (19%). One-, 2-, 5- and 10-year overall survival rates were 91%, 89%, 79% and 65%, respectively. A prognostic score, the CryoVas score (CVS), for the prediction of survival at 5 years was devised. Pulmonary and gastrointestinal involvement, glomerular filtration rate <60 ml/min and age >65 years were independently associated with death. At 5 years the death rates were 2.6%, 13.1%, 29.6% and 38.5% for a CVS of 0, 1, 2 and ≥3, respectively. At 1 year the death rates were 0%, 3.2%, 18.5% and 30.8% for a CVS of 0, 1, 2 and ≥3, respectively. The CVS was strongly correlated with the Five Factor Score (FFS) 2009, another prognostic score validated in primary necrotising vasculitis (r=0.82; p<0.0001). The area under the curve for the CVS was 0.74 compared with 0.67 for the FFS, indicating a better performance of the CVS (p=0.052).

    Conclusions In patients with non-infectious mixed CryoVas, the main prognostic factors are age >65 years, pulmonary and gastrointestinal involvement and renal failure. A score including these variables is significantly associated with the prognosis.

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    Footnotes

    • Competing interests None.

    • Ethics approval Ethics approval was obtained from Pitié-Salpétrière Institutional Review Board.

    • Provenance and peer review Not commissioned; externally peer reviewed.

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