title: Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey.
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.
authors.affiliation: Department of Internal Medicine, Cochin Hospital, Paris 75014, France. benjamin.terrier@cch.aphp.fr
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.forename: Fabrice
authors.initials: F
authors.lastname: Carrat
authors.forename: Evguenia
authors.initials: E
authors.lastname: Krastinova
authors.forename: Isabelle
authors.initials: I
authors.lastname: Marie
authors.forename: David
authors.initials: D
authors.lastname: Launay
authors.forename: Adeline
authors.initials: A
authors.lastname: Lacraz
authors.forename: Pauline
authors.initials: P
authors.lastname: Belenotti
authors.forename: Luc
authors.initials: L
authors.lastname: de Saint Martin
authors.forename: Thomas
authors.initials: T
authors.lastname: Quemeneur
authors.forename: Antoine
authors.initials: A
authors.lastname: Huart
authors.forename: Fabrice
authors.initials: F
authors.lastname: Bonnet
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.forename: Olivier
authors.initials: O
authors.lastname: Hinschberger
authors.forename: Patricia
authors.initials: P
authors.lastname: Rullier
authors.forename: Aurelie
authors.initials: A
authors.lastname: Hummel
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Lzaro
authors.forename: Frank
authors.initials: F
authors.lastname: Bridoux
authors.forename: Thierry
authors.initials: T
authors.lastname: Zenone
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Leger
authors.forename: Xavier
authors.initials: X
authors.lastname: Mariette
authors.forename: Patricia
authors.initials: P
authors.lastname: Senet
authors.forename: Emmanuelle
authors.initials: E
authors.lastname: Plaisier
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000368:Aged; D019540:Area Under Curve; D003449:Cryoglobulinemia / Q000150:complications / Q000401:mortality*; D003625:Data Collection; D005260:Female; D030342:Genetic Diseases, Inborn / Q000150:complications / Q000401:mortality*; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014657:Vasculitis / Q000209:etiology / Q000401:mortality*