Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey. Issue 3 (14th May 2012)

• Record Type: Journal Article
• Authors: 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
• Other Names: Laurent Aaron author non-byline.; Sébastien Abad author non-byline.; Redouane Bakir author non-byline.; Pauline Belenotti author non-byline.; Lucas Benarous author non-byline.; Nathalie Beneton author non-byline.; Gilles Blaison author non-byline.; Claire Blanchard-Delaunay author non-byline.; Fabrice Bonnet author non-byline.; Frank Bridoux author non-byline.; Patrice Cacoub author non-byline.; Pascal Cathébras author non-byline.; Fabrice Carrat author non-byline.; Laurent Chiche author non-byline.; Olivier Chosidow author non-byline.; Bernard Combe author non-byline.; Christian Combe author non-byline.; Nathalie Costedoat-Chalumeau author non-byline.; Pierre Cougoul author non-byline.; Bernard Cribier author non-byline.; Gilles Defuentes author non-byline.; Elisabeth Diot author non-byline.; Isabelle Durieu author non-byline.; Philippe Evon author non-byline.; Thibault Fraisse author non-byline.; Camille Francès author non-byline.; Hélène Francois author non-byline.; Thierry Frouget author non-byline.; Loik Geffray author non-byline.; Helder Gil author non-byline.; Jean Gutnecht author non-byline.; Eric Hachulla author non-byline.; Olivier Hermine author non-byline.; Olivier Hinschberger author non-byline.; Arnaud Hot author non-byline.; Antoine Huart author non-byline.; Aurélie Hummel author non-byline.; Jean-Emmanuel Kahn author non-byline.; Gilles Kaplanski author non-byline.; Alexandre Karras author non-byline.; Evguenia Krastinova author non-byline.; Adeline Lacraz author non-byline.; Claire Larroche author non-byline.; David Launay author non-byline.; Estibaliz Lazaro author non-byline.; Guillaume Le Guenno author non-byline.; Véronique Leblond author non-byline.; Jean-Marc Léger author non-byline.; Nicolas Limal author non-byline.; Eric Liozon author non-byline.; Catherine Lok author non-byline.; Véronique Loustau-Ratti author non-byline.; Kim Ly author non-byline.; Hervé Maillard author non-byline.; Raifah Makdassi author non-byline.; Isabelle Marie author non-byline.; Xavier Mariette author non-byline.; Olivier Meyer author non-byline.; Catherine Michel author non-byline.; Philippe Modiano author non-byline.; David Noel author non-byline.; Raphaele Nove-Josserand author non-byline.; Céline Onno author non-byline.; Christian Pagnoux author non-byline.; Caroline Pajot author non-byline.; Géraldine Perceau author non-byline.; Antoinette Perlat author non-byline.; Anne-Marie Piette author non-byline.; Emmanuelle Plaisier author non-byline.; Jean-François Pouget-Abadie author non-byline.; Thomas Quemeneur author non-byline.; Viviane Queyrel author non-byline.; Joseph Rivalan author non-byline.; Eric Rondeau author non-byline.; Patricia Rullier author non-byline.; Luc de Saint-Martin author non-byline.; Elisabeth Salles-Thomasson author non-byline.; Françoise Sarrot-Reynauld author non-byline.; Thierry Schaeverbeke author non-byline.; Gwendoline Sebille author non-byline.; Patricia Senet author non-byline.; Raphaele Seror author non-byline.; Jacques Serratrice author non-byline.; Alexandre Somogyi author non-byline.; Pascale Soria author non-byline.; Benjamin Terrier author non-byline.; Marie-Francoise Thiercelin-Legrand author non-byline.; François Truchetet author non-byline.; Jean-Paul Vagneur author non-byline.; Philippe Vanhille author non-byline.; Bérengère Vivet author non-byline.; Pierre-Jean Weiller author non-byline.; Bernadette Woehl-Kremer author non-byline.; Jean-Marie Woehl author non-byline.; Thierry Zénone author non-byline.;
• Is Part Of: Annals of the rheumatic diseases. Volume 72:Issue 3(2013)
• Page Start: 374
• Page End: 380
• Publication Date: 2012-05-14
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2012-201405
• Languages: English
• ISSNs: 0003-4967

Abstract

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.