Luc Mouthon1, Patrick H Carpentier1, Catherine Lok1, Pierre Clerson1, Virginie Gressin1, Eric Hachulla1, Alice Bérezné1, Elisabeth Diot1, Aurélie Khau Van Kien1, Patrick Jego1, Christian Agard1, Anne Bénédicte Duval-Modeste1, Agnès Sparsa1, Eve Puzenat1, Marie-Aleth Richard1. 1. From the Université Paris Descartes, Faculté de Médecine, Service de Médecine interne, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Paris; Service de Médecine vasculaire, CHU de Grenoble, Grenoble; Service de Dermatologie, Hôpital Sud, CHU Amiens, Amiens; Orgamétrie, Roubaix; Actelion Pharmaceuticals France, Paris; Service de Médecine interne, Centre de référence pour la sclérodermie systémique, Hôpital Claude Huriez, Université Lille 2, Lille; Service de Médecine interne, Hôpital Bretonneau, Tours; Service de Médecine interne, Hôpital Saint Eloi, Montpellier; Service de Médecine interne, Hôpital Sud, Rennes; Service de Médecine Interne, Centre Hospitalier Universitaire Hôtel Dieu, Nantes; Service de Dermatologie, Hôpital Charles Nicolle, Centre Hospitalier Universitaire, Rouen; Service de Dermatologie, Hôpital Dupuytren, CHU Limoges; Hôpital Saint Jacques, Besançon; Service de Dermatologie, Université Aix-Marseille, UMR 911, INSERM CRO2 UMR 911, Centre de recherche en oncologie biologique et oncopharmacologie, Hôpital de la Timone, Assistance Publique Hôpitaux de Marseille, Marseille, France.L. Mouthon, MD, PhD, Deputy Chief of Internal Medicine Department, Université Paris Descartes, Faculté de Médecine, Service de Médecine interne, Centre de référence pour les vascularites nécrosantes et la sclérodermie systémique, Hôpital Cochin, APHP; P.H. Carpentier, MD, Chief of Vascular Medicine Department, Service de Médecine vasculaire, CHU de Grenoble; C. Lok, MD, Chief of Dermatology Department, Service de Dermatologie, Hôpital Sud, CHU Amiens; P. Clerson, MD, Director, Orgamétrie; V. Gressin, MD, Medical Director, Actelion Pharmaceuticals France; E. Hachulla, MD, PhD, Deputy Chief of Internal Medicine Department, Service de Médecine interne, Centre de référence pour la sclérodermie systémique, Hôpital Claude Huriez,
Abstract
OBJECTIVE: Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc. METHODS: The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion. RESULTS: There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively. CONCLUSION: DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.
OBJECTIVE:Ischemic digital ulcers (DU) are frequent and severe complications of systemic sclerosis (SSc). The purpose of our study was to assess the effect of DU on hand disability and pain in patients with SSc. METHODS: The Evaluation of the Impact of Recurrent Ischemic DU on Hand Disability in Patients with SSc (ECLIPSE) is a prospective, multicenter, noninterventional study with a 2-year followup. Patients with SSc who experienced at least 1 DU in the previous year and received bosentan therapy were included between October 2009 and March 2011. This cohort is described at the time of inclusion. RESULTS: There were 190 patients (132 females) from 53 centers. Mean age ± SD was 43 ± 15 years at SSc diagnosis and 53 ± 15 years at inclusion. In 105 patients (56.2%), DU were the first non-Raynaud symptoms of SSc. The mean time interval between the occurrence of Raynaud phenomenon and the first DU episode was 6.6 ± 9.1 years. The mean numbers of active DU and fingers affected per patient for both hands were 2.3 ± 1.8 and 2.2 ± 1.6, respectively. Presence of active DU at inclusion was significantly associated with pain and impaired hand function: Visual Analog Scale for pain (0 to 10) was 6.2 ± 2.6 versus 2.5 ± 2.4 (p < 0.0001) and Cochin Hand Function Scale for hand disability (0 to 90) was 38 ± 20 versus 25 ± 19 (p < 0.0001), respectively. CONCLUSION:DU represent a major sign of SSc, often affecting multiple fingers and both hands. They are significantly associated with pain and hand disability.
Entities:
Keywords:
BOSENTAN; DIGITAL ULCERS; DISABILITY; HAND; SYSTEMIC SCLEROSIS
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