Digital ulcers in scleroderma: staging, characteristics and sub-setting through observation of 1614 digital lesions.
Laura Amanzi,Francesca Braschi,Ginevra Fiori,Felice Galluccio,I. Miniati,Serena Guiducci,Maria-Letizia Conforti,Olga Kaloudi,Francesca Nacci,Oana Sacu,Antonio Candelieri,Alberto Moggi Pignone,Laura Rasero,Domenico Conforti,Marco Matucci-Cerinic +14 more
TL;DR: In SSc, digital lesions are represented by DPS, DU, calcinosis and gangrene, and provide an evidence-based DU subsetting according to their origin and main characteristics.
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Abstract: Objective. To evaluate in SSc, the frequency of digital lesions and the morphology, characteristics, natural course and time to healing of 1614 digital ulcers (DUs). Methods. One hundred SSc patients were followed up for 4 years. In the first step, the digital lesions were observed and classified at the time of presentation [digital pitting scar (DPS); DU; calcinosis; gangrene]. In the second step, DUs were divided into subsets according to their origin and main features. In the third step, the time to healing was recorded for each DU and the influence of DU main characteristics on time to healing was also evaluated. Results. In the first step, 1614 digital lesions were observed: DPS, 712 (44.1%) lesions; DU, 785 (48.6%); calcinosis, 110 (6.8%); and gangrene, 7 (0.8%). In the second step, DUs were subsetted as follows: DU developed on DPS (8.8%), pure DU; DU developed on calcinosis (60%); DU derived from gangrene. In the third step, the mean time to healing was 25.6 (15.6) days in DPS, 76.2 (64) days in pure DU, 93.6 (59.2) days in calcinosis ulcers and 281.1 (263.3) in gangrene. Conclusions. In SSc, digital lesions are represented by DPS, DU, calcinosis and gangrene, and provide an evidence-based DU subsetting according to their origin and main characteristics. Subsetting may be helpful for a precise DU evaluation and staging, and in randomized controlled trials for a precise identification of those DUs that are to be included in therapeutic studies.
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Citations
Classification, categorization and essential items for digital ulcer evaluation in systemic sclerosis: a DeSScipher/European Scleroderma Trials and Research group (EUSTAR) survey
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Edoardo Rosato,Antonietta Gigante,Chiara Pellicano,A Colalillo,Danilo Alunni Fegatelli,Maurizio Muscaritoli +5 more
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Anti-annexin V autoantibodies and vascular abnormalities in systemic sclerosis: a longitudinal study.
Alex Magno Coelho Horimoto,Laize Guerreiro de Jesus,Albert Schiaveto de Souza,Silvia Helena Rodrigues,Cristiane Kayser +4 more
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Digital ulcers in systemic sclerosis: prevention by treatment with bosentan, an oral endothelin receptor antagonist.
Joseph H. Korn,Maureen D. Mayes,M. Matucci Cerinic,Maurizio Rainisio,Janet E. Pope,Eric Hachulla,Eric Rich,Patrick H. Carpentier,Jerry A. Molitor,James R. Seibold,Vivien Hsu,Loïc Guillevin,Sharmila Chatterjee,Hans-Hartmut Peter,J. Coppock,Ariane L. Herrick,Peter A. Merkel,Robert W. Simms,Christopher P. Denton,Daniel E. Furst,N. Nguyen,M. Gaitonde,Carol M. Black +22 more
TL;DR: Endothelins may play an important role in the pathogenesis of vascular disease in patients with SSc and treatment with bosentan may be effective in preventing new digital ulcers and improving hand function.
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Digital ulcers: overt vascular disease in systemic sclerosis
TL;DR: The recent approval (in Europe) of the dual endothelin receptor antagonist, bosentan, to reduce the number of new DUs in patients with SSc and ongoing DU disease, means that there is now an approved therapy--and new hope--for the treatment of D Us in these severely afflicted patients.
•Journal Article
Natural history of ischemic digital ulcers in systemic sclerosis: single-center retrospective longitudinal study.
Eric Hachulla,Pierre Clerson,David Launay,Marc Lambert,Sandrine Morell-Dubois,Viviane Queyrel,Pierre-Yves Hatron +6 more
TL;DR: Patients who are young at first sign of SSc, with high Rodnan scores and without vasodilator therapy, are at high risk of developing DU.
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