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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.

Benjamin Terrier 1, 2 Fabrice Carrat 3 Evguenia Krastinova 4 Isabelle Marie 5, 6 David Launay 7 Adeline Lacraz 8 Pauline Belenotti 9 Luc de Saint Martin 10, 11 Thomas Quemeneur 12 Antoine Huart 13 Fabrice Bonnet 14 Guillaume Le Guenno 15 Jean-Emmanuel Kahn 16 Olivier Hinschberger 17 Patricia Rullier 18 Aurelie Hummel Elisabeth Diot 19 Christian Pagnoux 20 Estibaliz Lzaro Franck Bridoux 21, 22 Thierry Zenone 23 Olivier Hermine 24 Jean-Marc Leger 25 Xavier Mariette 26 Patricia Senet 27 Emmanuelle Plaisier 28 Patrice Cacoub 29, 30
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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Submitted on : Friday, January 24, 2014 - 11:05:25 AM
Last modification on : Friday, May 20, 2022 - 11:06:04 AM

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Benjamin Terrier, Fabrice Carrat, Evguenia Krastinova, Isabelle Marie, David Launay, et al.. Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey.. Annals of the Rheumatic Diseases, BMJ Publishing Group, 2013, 72 (3), pp.374-80. ⟨10.1136/annrheumdis-2012-201405⟩. ⟨hal-00935851⟩

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