title: Update on the profile of the EUSTAR cohort: an analysis of the EULAR Scleroderma Trials and Research group database.
abstract: OBJECTIVE
  Systemic sclerosis (SSc) is a rare disease requiring multicentre collaboration to reveal comprehensive details of disease-related causes for morbidity and mortality.
  
  
  METHODS
  The European League Against Rheumatism (EULAR) Scleroderma Trials and Research (EUSTAR) group initiated a database to prospectively gather key data of patients with SSc using a minimal essential dataset that was reorganised in 2008 introducing new items. Baseline visit data of patients who were registered between 2004 and 2011 were analysed using descriptive statistics.
  
  
  RESULTS
  In June 2011, 7655 patients (2838 with diffuse cutaneous (dc) and 4481 with limited cutaneous (lc) SSc who fulfilled the American College of Rheumatology diagnostic criteria had been registered in 174 centres, mainly European. The most prominent hallmarks of disease were Raynaud's phenomenon (96.3%), antinuclear antibodies (93.4%) and a typical capillaroscopic pattern (90.9%). Scleroderma was more common on fingers and hands than on any other part of the skin. Proton pump inhibitors (65.2%), calcium channel blockers (52.7%), and corticosteroids (45.3%) were most often prescribed. Among the immunosuppressant agents, cyclophosphamide was used more often in dcSSc than in lcSSc.
  
  
  CONCLUSIONS
  The EUSTAR database provides an abundance of information on the true clinical face of SSc that will be helpful in improving the classification of SSc and its subsets and for developing more specific therapeutic recommendations.
authors.affiliation: Department of Internal Medicine and Rheumatology, Justus-Liebig-University Gießen Kerckhoff-Klinik, Bad Nauheim, Germany.
authors.forename: Florian M P
authors.initials: FM
authors.lastname: Meier
authors.forename: Klaus W
authors.initials: KW
authors.lastname: Frommer
authors.forename: Robert
authors.initials: R
authors.lastname: Dinser
authors.forename: Ulrich A
authors.initials: UA
authors.lastname: Walker
authors.forename: Laszlo
authors.initials: L
authors.lastname: Czirjak
authors.forename: Christopher P
authors.initials: CP
authors.lastname: Denton
authors.forename: Yannick
authors.initials: Y
authors.lastname: Allanore
authors.forename: Oliver
authors.initials: O
authors.lastname: Distler
authors.forename: Gabriela
authors.initials: G
authors.lastname: Riemekasten
authors.forename: Gabriele
authors.initials: G
authors.lastname: Valentini
authors.forename: Ulf
authors.initials: U
authors.lastname: Müller-Ladner
mesh_terms: D000974:Antibodies, Antinuclear / Q000097:blood; D002121:Calcium Channel Blockers / Q000627:therapeutic use; D015331:Cohort Studies; D003520:Cyclophosphamide / Q000627:therapeutic use; D016208:Databases, Factual; D005060:Europe; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D054328:Proton Pump Inhibitors / Q000627:therapeutic use; D011928:Raynaud Disease / Q000150:complications / Q000175:diagnosis; D045743:Scleroderma, Diffuse / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D045745:Scleroderma, Limited / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology
chemical_list: D000974:Antibodies, Antinuclear; D002121:Calcium Channel Blockers; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D054328:Proton Pump Inhibitors; D003520:Cyclophosphamide