Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey.

Benjamin Terrier; Fabrice Carrat; Evguenia Krastinova; Isabelle Marie; David Launay; Adeline Lacraz; Pauline Belenotti; Luc De Saint Martin; Thomas Quemeneur; Antoine Huart; Fabrice Bonnet; Guillaume Le Guenno; Jean-Emmanuel Kahn; Olivier Hinschberger; Patricia Rullier; Aurelie Hummel; Elisabeth Diot; Christian Pagnoux; Estibaliz Lzaro; Franck Bridoux; Thierry Zenone; Olivier Hermine; Jean-Marc Leger; Xavier Mariette; Patricia Senet; Emmanuelle Plaisier; Patrice Cacoub

doi:10.1136/annrheumdis-2012-201405

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}

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 [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 de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse

14 Foie, métabolismes et cancer

15 Centre Hospitalier Universitaire Estaing

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 Service de neurologie 1 [CHU Pitié-Salpétrière]

26 Régulation de la réponse immune, infection VIH-1 et autoimmunité

27 Service de Dermatologie [CHU Tenon]

28 Remodelage et Reparation du Tissu Renal

29 Service de médecine interne [CHU Pitié-Salpétrière]

30 BTPI - Biologie et thérapeutique des pathologies immunitaires

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.

Type de document :

Article dans une revue

Annals of the Rheumatic Diseases, BMJ Publishing Group, 2013, 72 (3), pp.374-80. 〈10.1136/annrheumdis-2012-201405〉

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Sciences du Vivant [q-bio]

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http://hal.univ-brest.fr/hal-00935851
Contributeur : Ghislaine Calvez <>
Soumis le : vendredi 24 janvier 2014 - 11:05:25
Dernière modification le : vendredi 27 juillet 2018 - 15:04:02

Prognostic factors of survival in patients with non-infectious mixed cryoglobulinaemia vasculitis: data from 242 cases included in the CryoVas survey.

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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.

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