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205. THU0237 Impact of recurrent ischemic digital ulcers on hand disability in systemic sclerosis –

Author

Agnès Sparsa, Eve Puzenat, Virginie Gressin, Alice Bérezné, Marie-Aleth Richard, Pierre Clerson, L. Mouthon, A. Khau Van Kien, E. Hachulla, Camille Francès, Hervé Maillard, P.-H. Carpentier, Catherine Lok, and E. Diot

Subjects
Gangrene, medicine.medical_specialty, business.industry, Visual analogue scale, medicine.medical_treatment, Immunology, Interim analysis, medicine.disease, General Biochemistry, Genetics and Molecular Biology, Bosentan, Surgery, Rheumatology, Quality of life, Amputation, Concomitant, Internal medicine, Immunology and Allergy, Medicine, Complication, business, medicine.drug
Abstract

Background Ischemic digital ulcers (DU) are a common complication of systemic sclerosis (SSc). Objectives To evaluate the impact of recurrent DU on hand disability in a prospective, longitudinal, observational study of 24 months. Methods Patients fulfilled ACR or Leroy-Medsger criteria for SSc, experienced at least one DU during the previous year with or without active DU at inclusion, and were eligible for bosentan therapy. Data were collected on SSc, characteristics of past and present DU, modified Rodnan skin score, factors influencing hand function and mobility, disability scores (Cochin hand function scale - CHFS, health assessment questionnaire disability index - HAQ-DI), and quality of life (SF36). Results 217 patients were included between October 2009 and March 2011 in 50 centers. The interim analysis includes 184 patients (128 women). Mean age was 43±15 years at diagnosis of SSc and 53±15 years at inclusion. SSc was diffuse in 44% of patients and Raynaud’s phenomenon (RP) started 14±12 years before inclusion. 11% of patients had pulmonary arterial hypertension. Mean Rodnan score was 14.3±8.8. Time elapsed since the first DU was 6±7 years. In 47% of patients, DU was an early complication (first non-RP sign) and 59% had recurrent DU. Sequelae of DU included loss of substance (62%), autoamputation (8%), and surgical amputation (10%). Complications were infection (8%), gangrene (5%), and osteitis (2%). 54% of patients had at least one active DU at inclusion. In these patients, the mean number of DU was 2.3±1.9; 54% had more than one DU, 37% had both hands involved, and on average 2.2±1.7 fingers were affected. 21% of cases had at least one extended DU (>1cm). Concomitant mechanical ulcers were localized at the dorsal face of fingers (19%), bony reliefs (13%), or calcinosis (1%). DU were painful (visual analogue scale, 6.18±2.56 vs. 2.49±2.56 without DU) and disabling, involving the dominant hand in 72% of patients. DU worsened hand disability (median CHFS, 42 with active DU vs. 26 without DU, p Conclusions DU represent an early complication of SSc. They are painful, affecting often multiple fingers and both hands. In patients with SSc, DU are significantly associated with hand disability. Prospective follow-up at two years will allow to further elucidate the specific role that DU episodes play in the disability of these patients. Disclosure of Interest L. Mouthon Consultant for: Lilly, Actelion France, GSK, Pfizer, P. Carpentier Consultant for: Actelion, A. Khau Van Kien: None Declared, P. Clerson: None Declared, H. Maillard: None Declared, E. Hachulla Consultant for: Actelion, GSK, Pfizer, C. Frances: None Declared, E. Diot: None Declared, C. Lok Consultant for: Actelion, E. Puzenat Consultant for: Actelion, A. Sparsa Consultant for: Actelion, A. Berezne: None Declared, V. Gressin Employee of: Actelion France, M.-A. Richard Consultant for: Actelion

Published
2013
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206. FRI0253 Use of bosentan for the prevention of digital ulcers related to systemic sclerosis. Retrospective french study of 89 patients treated since 2007

Author

Christian Agard, Alice Bérezné, Isabelle Quéré, Jean Sibilia, A. Khau Van Kien, Emmanuel Chatelus, E. Hachulla, Patrick Carpentier, Luc Mouthon, Pierre Clerson, and Virginie Gressin

Subjects
medicine.medical_specialty, business.industry, Immunology, Retrospective cohort study, Disease, General Biochemistry, Genetics and Molecular Biology, Bosentan, respiratory tract diseases, National cohort, Clinical trial, Rheumatology, Internal medicine, medicine, Physical therapy, Diffuse disease, Immunology and Allergy, Dosing, Active smoking, business, medicine.drug
Abstract

Background Bosentan, an endothelin-1 receptor antagonist, is indicated in Europe since 2007 for the prevention of new digital ulcers (DU) in systemic sclerosis (SSc). Objectives To identify modalities of bosentan use and the profile of patients receiving such preventive treatment at any time for any duration since 2007 in current practice. Methods This retrospective observational study was conducted in 2011 in 10 French specialist centers. Patients included were adults with SSc according to ACR or Leroy-Medsger criteria, starting bosentan for prevention of DU between 2007 and 2010. Patients treated with bosentan in clinical trials and patients deceased since the start of bosentan were excluded. From the complete list of eligible patients of each center, up to 10 patients were randomly selected. Results 89 patients (mean age, 52 years) were drawn from 130 eligible patients. Male-to-female ratio was 1/2.2. SSc was diffuse in 44% of patients; 26% had continuous corticoid therapy; 1 out of 4 was active smoker. At start of bosentan, Raynaud’s phenomenon (RP) was present for 14.7±12 years. Time since first DU episode was 6.5±7 years, but 42% of patients had recent ulcerative disease ( Conclusions This study has created the first national cohort of patients treated with bosentan for prevention of DU in SSc in France. Patients treated with bosentan have severe ulcerative disease, which is often resistant to other preventive measures. Among these, male patients and those with diffuse disease forms appear overrepresented. One quarter of patients present the potentially aggravating factors corticoid therapy and active smoking. In the large majority of cases target dosing of bosentan was respected; tolerance was satisfactory and comparable to results of previous studies. Disclosure of Interest C. Agard Consultant for: Actelion°, P. Carpentier Consultant for: Actelion°, L. Mouthon Grant/Research support from: Actelion°, Consultant for: Actelion°, P. Clerson Consultant for: Actelion°, V. Gressin Employee of: Actelion°, A. Berezne: None Declared, E. Chatelus: None Declared, A. Khau Van Kien: None Declared, I. Quere: None Declared, J. Sibilia: None Declared, E. Hachulla Grant/Research support from: Actelion°, Consultant for: Actelion°, Speakers Bureau: Actelion°

Published
2013
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210. Standards de qualité pour la pratique de l’examen écho-doppler dans l’étude des malformations et tumeurs vasculaires. Rapport de la Société française de médecine vasculaire (SFMV)

Author

Laroche, J.-P., primary, Becker, F., additional, Khau-Van-Kien, A., additional, Baudoin, P., additional, Brisot, D., additional, Buffler, A., additional, Coupé, M., additional, Jurus, C., additional, Mestre, S., additional, Miserey, G., additional, Soulier-Sotto, V., additional, Tissot, A., additional, Viard, A., additional, Vignes, S., additional, and Quéré, I., additional

Published
2013
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212. Relation entre le phénotype clinique et les anomalies du stress oxydant et de la prolifération cellulaire observées au cours de la sclérodermie systémique et de la maladie de Rendu-Osler

Author

Guilpain, P., primary, Cerutti, D., additional, Riviere, S., additional, Kavian, N., additional, Khau Van Kien-Wautot, A., additional, Quere, I., additional, Vincent, T., additional, Borderie, D., additional, Batteux, F., additional, and Le Quellec, A., additional

Published
2012
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213. Jusqu’où aller avec l’écho-Doppler ?

Author

Laroche, J.-P., primary, Khau Van Kien, A., additional, Galanaud, J.-P., additional, Brisot, D., additional, Boge, G., additional, Tapon, M., additional, Zapulla, C., additional, Raczka, V., additional, Perez-Martin, A., additional, Schuster, I., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2012
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214. Artériopathie chronique oblitérante des membres inférieurs (AOMI) : prévention primaire, prévention secondaire. Le point sur les recommandations

Author

Laroche, J.-P., primary, Khau Van Kien, A., additional, Galanaud, J.-P., additional, Brisot, D., additional, Boge, G., additional, Tapon, M., additional, Zapulla, C., additional, Raczka, V., additional, Perez-Martin, A., additional, Schuster, I., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2012
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215. Embolie pulmonaire asymptomatique dans le syndrome de Churg et Strauss

Author

Maria, A., primary, Guilpain, P., additional, Forestier, A., additional, Delhom, E., additional, Schiffmann, A., additional, Riviere, S., additional, Khau Van Kien, A., additional, Leray-Moragues, H., additional, Serre, I., additional, Vincent, T., additional, Eliaou, J.F., additional, and Le Quellec, A., additional

Published
2012
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217. Toute thrombose veineuse profonde (TVP) doit marcher

Author

Laroche, J.-P., primary, Khau Van Kien, A., additional, Galanaud, J.-P., additional, Brisot, D., additional, Böge, G., additional, Tapon, M., additional, Zapulla, C., additional, Raczka, V., additional, Perez-Martin, A., additional, Schuster, I., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2012
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218. Techniciens de santé ou professions de santé intermédiaires : quelles applications pour la Médecine Vasculaire ?

Author

Laroche, J.-P., primary, Becker, F., additional, Khau Van Kien, A., additional, Galanaud, J.-P., additional, Brisot, D., additional, Böge, G., additional, Tapon, M., additional, Zapulla, C., additional, Raczka, V., additional, Perez-Martin, A., additional, Schuster, I., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2012
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219. L’ulcère digital ischémique, complication fréquente et sévère de la sclérodermie systémique. Les données d’inclusion de la cohorte ECLIPSE

Author

Richard, M.-A., primary, Carpentier, P., additional, Clerson, P., additional, Lok, C., additional, Puzenat, E., additional, Gressin, V., additional, Hachulla, E., additional, Bérezné, A., additional, Diot, E., additional, Khau Van Kien, A., additional, Sparsa, A., additional, Frances, C., additional, Maillard, H., additional, and Mouthon, L., additional

Published
2012
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221. S.11.1 Influence of digital ulcer healing on disability and daily activity limitations in SSc

Author

Berezne, A., primary, Seror, R., additional, Bussone, G., additional, Nguyen, C., additional, Morell-Dubois, S., additional, Fois, E., additional, Guillevin, L., additional, Mouthon, L., additional, Carpentier, P., additional, Khau Van Kien, A., additional, Clerson, P., additional, Maillard, H., additional, Hachulla, E., additional, Frances, C., additional, Diot, E., additional, Lok, C., additional, Puzenat, E., additional, Sparsa, A., additional, Berezne, A., additional, Gressin, V., additional, Richard, M. A., additional, Saketkoo, L. A., additional, Escorpizo, R., additional, Keen, K., additional, Fligelstone, K., additional, Distler, O., additional, Assassi, S., additional, Leyva, A., additional, Mayes, M., additional, Sharif, R., additional, Nair, D., additional, Fischbach, M., additional, Nguyen, N., additional, Reveille, J., additional, Gonzalez, E., additional, McNearney, T., additional, Riccieri, V., additional, Sciarra, I., additional, Maset, L., additional, Passi, L., additional, Stefanantoni, K., additional, Vasile, M., additional, Scarno, A., additional, and Spadaro, A., additional

Published
2012
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223. Évaluation de l’impact de la survenue répétée d’ulcères digitaux ischémiques sur le handicap fonctionnel des mains de patients sclérodermiques (ECLIPSE). Description de la cohorte à l’inclusion

Author

Mouthon, L., primary, Carpentier, P., additional, Lok, C., additional, Clerson, P., additional, Puzenat, E., additional, Hachulla, E., additional, Frances, C., additional, Berezne, A., additional, Khau Van Kien-Wautot, A., additional, Diot, E., additional, Gressin, V., additional, and Richard, M.-A., additional

Published
2011
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224. Évaluation de l’impact de la survenue répétée d’ulcères digitaux ischémiques sur le handicap fonctionnel des mains de patients sclérodermiques (ECLIPSE). Description de la cohorte à l’inclusion

Author

Richard, M.-A., primary, Carpentier, P., additional, Clerson, P., additional, Lok, C., additional, Puzenat, E., additional, Gressin, V., additional, Hachulla, E., additional, Bérezné, A., additional, Diot, E., additional, Khau Van Kien, A., additional, Sparsa, A., additional, Frances, C., additional, Maillard, H., additional, and Mouthon, L., additional

Published
2011
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225. Prévention des ulcères digitaux de la sclérodermie systémique par le bosentan. Étude française rétrospective de 89 patients traités depuis 2007

Author

Agard, C., primary, Carpentier, P., additional, Mouthon, L., additional, Clerson, P., additional, Gressin, V., additional, Diot, E., additional, Jego, P., additional, Lok, C., additional, Sparsa, A., additional, Khau Van Kien-Wautot, A., additional, Sibilia, J., additional, and Hachulla, E., additional

Published
2011
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226. Le syndrome de Cockett : mythe ou réalité ?

Author

Laroche, J.-P., primary, Becker, J.-F., additional, Dadon, M., additional, Brisot, D., additional, Coupé, M., additional, Khau Van Kien, A., additional, Böge, G., additional, Galanaud, J.-P., additional, Perez Martin, A., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2011
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228. Genotype-phenotype correlation in 104 patients with vascular Ehlers-Danlos syndrome: Evidence for a mild form of the disease

Author

Bal, L., primary, Fauret, A.-L., additional, Ranque, B., additional, Frank, M., additional, Golmard, L., additional, Travers, C., additional, Khau Van Kien, P., additional, Denarié, N., additional, Messas, E., additional, Plauchu, H., additional, Germain, D., additional, Emmerich, J., additional, Fiessinger, J.-N., additional, and Jeunemaître, X., additional

Published
2011
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233. Banques de données de mutations : enjeux et perspectives pour les maladies génétiques orphelines

Author

Humbertclaude, V., primary, Tuffery-Giraud, S., additional, Bareil, C., additional, Thèze, C., additional, Paulet, D., additional, Desmet, F.-O., additional, Hamroun, D., additional, Baux, D., additional, Girardet, A., additional, Collod-Béroud, G., additional, Khau Van Kien, P., additional, Roux, A.-F., additional, des Georges, M., additional, Béroud, C., additional, and Claustres, M., additional

Published
2010
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234. Complications et resténose d’angioplastie avec stent iliaque. Comparatif écho-Doppler angioscanner. Intérêt de l’IPS

Author

Laroche, J.-P., primary, Brisot, D., additional, Coupé, M., additional, Khau Van Kien, A., additional, Zappula, C., additional, Böge, G., additional, Raczka, V., additional, Galanaud, J.-P., additional, Soulier Sotto, V., additional, Tapon, M., additional, Perez Martin, A., additional, Schuster, I., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2010
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240. Intérêt du dépistage de l’artériopathie oblitérante des membres inférieurs asymptomatique

Author

Laroche, J.-P., primary, Khau Van Kien, A., additional, Galanaud, J.-P., additional, Brisot, D., additional, Böge, G., additional, Perez-Martin, A., additional, Dauzat, M., additional, Schuster, I., additional, Carpentier, P., additional, Bosson, J.-L., additional, Rolland, C., additional, Becker, F., additional, Constans, J., additional, and Quéré, I., additional

Published
2010
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241. L’angiosonologie vasculaire, concept, niveaux d’expertise

Author

Dauzat, M., primary, Laroche, J.-P., additional, Brisot, D., additional, Coupé, M., additional, Khau Van Kien, A., additional, Zappula, C., additional, Böge, G., additional, Raczka, V., additional, Galanaud, J.-P., additional, Soulier Sotto, V., additional, Tapon, M., additional, Perez Martin, A., additional, Schuster, I., additional, and Quéré, I., additional

Published
2010
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242. Évaluation de l’impact de la survenue répétée d’ulcères digitaux ischémiques sur le handicap fonctionnel des mains de patients sclérodermiques (ECLIPSE). Description de la cohorte à l’inclusion

Author

Hervé Maillard, Camille Francès, Alice Bérezné, P.-H. Carpentier, Eric Hachulla, A. Khau Van Kien, Agnès Sparsa, Elisabeth Diot, Catherine Lok, Luc Mouthon, Eve Puzenat, Marie-Aleth Richard, Virginie Gressin, and Pierre Clerson

Subjects
Gynecology, medicine.medical_specialty, business.industry, Medicine, Dermatology, business
Published
2011
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243. Évaluation de l’impact de la survenue répétée d’ulcères digitaux ischémiques sur le handicap fonctionnel des mains de patients sclérodermiques (ECLIPSE). Description de la cohorte à l’inclusion

Author

A. Khau Van Kien-Wautot, Catherine Lok, Eve Puzenat, Pierre Clerson, Camille Francès, L. Mouthon, M.-A. Richard, E. Diot, E. Hachulla, Patrick H. Carpentier, Alice Bérezné, and Virginie Gressin

Subjects
Gastroenterology, Internal Medicine
Published
2011
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244. Genotype-phenotype correlation in 104 patients with vascular Ehlers-Danlos syndrome: Evidence for a mild form of the disease

Author

Jean-Noël Fiessinger, Dominique P. Germain, Xavier Jeunemaitre, Emmanuel Messas, P. Khau Van Kien, Joseph Emmerich, Anne-Laure Fauret, Brigitte Ranque, Lisa Golmard, C. Travers, L. Bal, Henri Plauchu, M. Frank, and Nicolas Denarié

Subjects
Genetics, medicine.medical_specialty, business.industry, Ehlers–Danlos syndrome, Genetic counseling, Medicine, Mild form, Disease, Cardiology and Cardiovascular Medicine, business, medicine.disease, Dermatology, Genotype phenotype
Published
2011
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245. Comparison of Clinical Presentations and Outcomes Between Patients With TGFBR2 and FBN1 Mutations in Marfan Syndrome and Related Disorders

Author

Attias, David, primary, Stheneur, Chantal, additional, Roy, Carine, additional, Collod-Béroud, Gwenaëlle, additional, Detaint, Delphine, additional, Faivre, Laurence, additional, Delrue, Marie-Ange, additional, Cohen, Laurence, additional, Francannet, Christine, additional, Béroud, Christophe, additional, Claustres, Mireille, additional, Iserin, Franck, additional, Khau Van Kien, Philippe, additional, Lacombe, Didier, additional, Le Merrer, Martine, additional, Lyonnet, Stanislas, additional, Odent, Sylvie, additional, Plauchu, Henri, additional, Rio, Marlène, additional, Rossi, Annick, additional, Sidi, Daniel, additional, Steg, Philippe Gabriel, additional, Ravaud, Philippe, additional, Boileau, Catherine, additional, and Jondeau, Guillaume, additional

Published
2009
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249. Profil des patients candidats à une hospitalisation

Author

Laroche, J.-P., primary, Brisot, D., additional, Coupé, M., additional, Khau Van Kien, A., additional, Zappula, C., additional, Böge, G., additional, Raczka, V., additional, Galanaud, J.-P., additional, Schuster, I., additional, Soulier Sotto, V., additional, Dauzat, M., additional, and Quéré, I., additional

Published
2009
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