학술논문
Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey
Document Type
Academic Journal
Author
Terrier, Benjamin; Carrat, Fabrice; Krastinova, Evguenia; Marie, Isabelle; Launay, David; Lacraz, Adeline; Belenotti, Pauline; de Saint Martin, Luc; Quemeneur, Thomas; Huart, Antoine; Bonnet, Fabrice; Le Guenno, Guillaume; Kahn, Jean-Emmanuel; Hinschberger, Olivier; Rullier, Patricia; Hummel, Aurelie; Diot, Elisabeth; Pagnoux, Christian; Lzaro, Estibaliz; Bridoux, Frank; Zenone, Thierry; Hermine, Olivier; Leger, Jean-Marc; Mariette, Xavier; Senet, Patricia; Plaisier, Emmanuelle; Cacoub, Patrice; Laurent, Aaron; Sébastien, Abad; Redouane, Bakir; Pauline, Belenotti; Lucas, Benarous; Nathalie, Beneton; Gilles, Blaison; Claire, Blanchard-Delaunay; Fabrice, Bonnet; Frank, Bridoux; Patrice, Cacoub; Pascal, Cathébras; Fabrice, Carrat; Laurent, Chiche; Olivier, Chosidow; Bernard, Combe; Christian, Combe; Nathalie, Costedoat-Chalumeau; Pierre, Cougoul; Bernard, Cribier; Gilles, Defuentes; Elisabeth, Diot; Isabelle, Durieu; Philippe, Evon; Thibault, Fraisse; Camille, Francès; Hélène, Francois; Thierry, Frouget; Loik, Geffray; Helder, Gil; Jean, Gutnecht; Eric, Hachulla; Olivier, Hermine; Olivier, Hinschberger; Arnaud, Hot; Antoine, Huart; Aurélie, Hummel; Jean-Emmanuel, Kahn; Gilles, Kaplanski; Alexandre, Karras; Evguenia, Krastinova; Adeline, Lacraz; Claire, Larroche; David, Launay; Estibaliz, Lazaro; Guillaume, Le Guenno; Véronique, Leblond; Jean-Marc, Léger; Nicolas, Limal; Eric, Liozon; Catherine, Lok; Véronique, Loustau-Ratti; Kim, Ly; Hervé, Maillard; Raifah, Makdassi; Isabelle, Marie; Xavier, Mariette; Olivier, Meyer; Catherine, Michel; Philippe, Modiano; David, Noel; Raphaele, Nove-Josserand; Céline, Onno; Christian, Pagnoux; Caroline, Pajot; Géraldine, Perceau; Antoinette, Perlat; Anne-Marie, Piette; Emmanuelle, Plaisier; Jean-François, Pouget-Abadie; Thomas, Quemeneur; Viviane, Queyrel; Joseph, Rivalan; Eric, Rondeau; Patricia, Rullier; Luc, de Saint-Martin; Elisabeth, Salles-Thomasson; Françoise, Sarrot-Reynauld; Thierry, Schaeverbeke; Gwendoline, Sebille; Patricia, Senet; Raphaele, Seror; Jacques, Serratrice; Alexandre, Somogyi; Pascale, Soria; Benjamin, Terrier; Marie-Francoise, Thiercelin-Legrand; François, Truchetet; Jean-Paul, Vagneur; Philippe, Vanhille; Bérengère, Vivet; Pierre-Jean, Weiller; Bernadette, Woehl-Kremer; Jean-Marie, Woehl; Thierry, Zénone
Source
Annals of Rheumatic Diseases. Mar 01, 2013 72(3):374-380
Subject
Language
English
ISSN
0003-4967
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.