title: An observational cohort study of patients with newly diagnosed digital ulcer disease secondary to systemic sclerosis registered in the EUSTAR database.
abstract: OBJECTIVE
  This study describes clinical characteristics, prognostic factors, and quality of life in patients with newly diagnosed (incident) digital ulcers (DU).
  
  
  METHODS
  Observational cohort study of 189 consecutive SSc patients with incident DU diagnosis identified from the EUSTAR database (22 centres in 10 countries). Data were collected from medical charts and during one prospective visit between 01/2004 and 09/2010.
  
  
  RESULTS
  Median age at DU diagnosis was 51 years, majority of patients were female (88%), and limited cutaneous SSc was the most common subtype (61%). At incident DU diagnosis, 41% of patients had one DU and 59% had ≥2 DU; at the prospective visit 52% had DU. Pulmonary arterial hypertension (PAH) and multiple DU at diagnosis were associated with presence of any DU at the prospective visit (odds ratios: 4.34 and 1.32). During the observation period (median follow-up was 2 years) 127 patients had ≥1 hospitalisation. The event rate of new DU per person-year was 0.66, of DU-associated complications was 0.10, and of surgical or diagnostic procedures was 0.12. At the prospective visit, patients with ≥1 DU reported impairment in daily activities by 57%, those with 0 DU by 37%. The mean difference between patients with or without DU in the SF-36 physical component was 2.2, and in the mental component 1.4. DU patients were not routinely prescribed endothelin receptor antagonists or prostanoids.
  
  
  CONCLUSIONS
  This real world cohort demonstrates that DU require hospital admission, and impair daily activity. PAH and multiple DU at diagnosis were associated with future occurrence of DU.
authors.affiliation: Actelion Pharmaceuticals Ltd., Departments of Epidemiology and Observational Studies, and Global Market Access and Pricing, Allschwil, Switzerland.
authors.forename: Monika
authors.initials: M
authors.lastname: Brand
authors.affiliation: Basel University Department of Rheumatology, Felix Platter-Spital, Basel, Switzerland.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Hollaender
authors.affiliation: Actelion Pharmaceuticals Ltd., Departments of Epidemiology and Observational Studies, and Global Market Access and Pricing, Allschwil, Switzerland.
authors.forename: Daniel
authors.initials: D
authors.lastname: Rosenberg
authors.affiliation: Numerus Statistics Ltd., Tübingen, Germany.
authors.forename: Martin
authors.initials: M
authors.lastname: Scott
authors.affiliation: Actelion Pharmaceuticals Ltd., Departments of Epidemiology and Observational Studies, and Global Market Access and Pricing, Allschwil, Switzerland.
authors.forename: Elke
authors.initials: E
authors.lastname: Hunsche
authors.affiliation: Basel University Department of Rheumatology, Felix Platter-Spital, Basel, Switzerland.
authors.forename: Alan
authors.initials: A
authors.lastname: Tyndall
authors.affiliation: Department of Biomedicine, Section of Rheumatology, University of Florence, Florence, Italy.
authors.forename: Valentina
authors.initials: V
authors.lastname: Denaro
authors.affiliation: Servicio de Reumatologia, Hospital Universitario 12 de Octubre, Madrid, Spain.
authors.forename: Patricia
authors.initials: P
authors.lastname: Carreira
authors.affiliation: Department of Rheumatology and Immunology, University of Pécs, Pécs, Hungary.
authors.forename: Cecilia
authors.initials: C
authors.lastname: Varju
authors.affiliation: Department Clinica Medica, Università Politecnica delle Marche, Ancona, Italy.
authors.forename: Barbara
authors.initials: B
authors.lastname: Gabrielli
authors.affiliation: Rheumatology and Clinical Immunology Service, Spedali Civili di Brescia, Brescia, Italy.
authors.forename: Stefania
authors.initials: S
authors.lastname: Zingarelli
authors.affiliation: Rheumatology Unit, University of Verona, Verona, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Caramaschi
authors.affiliation: Institute of Rheumatology, University of Belgrade Medical School, Belgrade, Serbia.
authors.forename: Katarina
authors.initials: K
authors.lastname: Simic-Pasalic
authors.affiliation: Department of Rheumatology, Justus- Liebig-University, Bad Nauheim, Germany.
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
authors.affiliation: Department of Internal Medicine and Medical Specialities, Sapienza University of Rome, Rome, Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Vasile
authors.affiliation: Department of Rheumatology, Cantacuzino Hospital, Carol Davila University, Bucharest, Romania.
authors.forename: Carina
authors.initials: C
authors.lastname: Mihai
authors.affiliation: Clinical Immunology Unit, Sapienza University of Rome, Rome, Italy.
authors.forename: Edoardo
authors.initials: E
authors.lastname: Rosato
authors.affiliation: AOU Rheumatology Unit, Department of Medical Sciences, University of Cagliari, Cagliari, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Vacca
authors.affiliation: Department of Internal Medicine, Centre Hospitalier, Valence, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Zenone
authors.affiliation: University Hospital, Alexandria, Egypt.
authors.forename: Walid A
authors.initials: WA
authors.lastname: Mohamed
authors.affiliation: Department of Rheumatology, Gr. T. Popa University of Medicine and Pharmacy, Iasi, Romania.
authors.forename: Codrina
authors.initials: C
authors.lastname: Ancuta
authors.affiliation: Department of Rheumatology, University of Genoa, Genoa, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Zampogna
authors.affiliation: University of Medicine and Pharmacy Iuliu Hatieganu, Cluj Napoca, Romania.
authors.forename: Simona
authors.initials: S
authors.lastname: Rednic
authors.affiliation: Division of Rheumatology, Chris Hani Baragwanath Academic Hospital, Johannesburg, South Africa.
authors.forename: Nadia
authors.initials: N
authors.lastname: Jabaar
authors.affiliation: Rheumatology Unit, Istituto Clinico Humanitas, University of Milan, Rozzano, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Belloli
authors.affiliation: Clinica Medica, Azienda Ospedaliera San Gerardo, University of Milano, Bicocca, Italy.
authors.forename: Maria R
authors.initials: MR
authors.lastname: Pozzi
authors.affiliation: Rheumatology Unit, Azienda Ospedaliera Universitaria Policlinico V.E., Catania, Italy.
authors.forename: Rosario
authors.initials: R
authors.lastname: Foti
authors.affiliation: Basel University Department of Rheumatology, Felix Platter-Spital, Basel, Switzerland. ulrich.walker@usb.ch.
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D017281:Cost of Illness; D016208:Databases, Factual; D005060:Europe / Q000453:epidemiology; D005260:Female; D005385:Fingers / Q000098:blood supply*; D006760:Hospitalization; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000453:epidemiology; D015994:Incidence; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D011379:Prognosis; D011446:Prospective Studies; D011788:Quality of Life; D012008:Recurrence; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D012883:Skin Ulcer / Q000175:diagnosis / Q000453:epidemiology* / Q000503:physiopathology / Q000523:psychology / Q000628:therapy; D013997:Time Factors