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 (25, 29)

1 I3 - Immunologie - Immunopathologie - Immunothérapeutique
2 Service de Médecine Interne
3 ESIM - Epidémiologie des maladies infectieuses et modélisation
4 CESP - Centre de recherche en épidémiologie et santé des populations
5 Service de Médecine Interne [CHU Rouen]
6 Physiopathologie et biothérapies des maladies inflammatoires et autoimmunes
7 Department of Internal Medicine
8 Service de Néphrologie-Transplantation-Dialyse
9 MARSEILLE - Med Int - Service de Médecine Interne
10 DMIP - Brest - Département de Médecine Interne et Pneumologie [Brest]
11 GETBO - Groupe d'Etude de la Thrombose de Bretagne Occidentale
12 VALENCIENNES - Med Int - Département de Médecine Interne
13 Service Médecine interne et immunopathologie clinique [CHU Toulouse]
14 Foie, métabolismes et cancer
15 CHU Clermont-Ferrand
16 EA 2686 - Laboratoire d'Immunologie
17 MULHOUSE - Med Int - Département de Médecine Interne
18 Service de Médecine Interne B
19 Service de Médecine Interne
20 Service de médecine interne et centre de référence des maladies rares [CHU Cochin]
21 CRIBL - Contrôle de la Réponse Immune B et des Lymphoproliférations
22 Service de néphrologie - hémodialyse et transplantation rénale
23 VALENCE - Med Int - Médecine Interne
24 CHRI - Cytokines, hématopoïèse et réponse immune
25 CHU Pitié-Salpêtrière [AP-HP]
26 Régulation de la réponse immune, infection VIH-1 et autoimmunité
27 CHU Tenon [AP-HP]
28 UMR S702 - Remodelage et Reparation du Tissu Renal
29 BTPI - Biologie et thérapeutique des pathologies immunitaires

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.

Domaines: Sciences du Vivant [q-bio]

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, 2013, 72 (3), pp.374-80. ⟨10.1136/annrheumdis-2012-201405⟩. ⟨hal-00935851⟩