title: Retrospective analysis of the frequency of centrofacial telangiectasia in systemic sclerosis patients treated with bosentan or ilomedin.
abstract: BACKGROUND
  Bosentan is a dual endothelin receptor antagonist initially introduced for the treatment of pulmonary arterial hypertension and recently approved for the treatment of digital ulcers in patients with systemic sclerosis (SSc). Our clinical observations indicate that bosentan therapy may be associated with an increased frequency of centrofacial telangiectasia (TAE). Here, we sought to analyze the frequency of TAE in patients with SSc who were treated with either bosentan or the prostacyclin analog iloprost.
  
  
  METHODS
  We conducted a retrospective analysis in 27 patients with SSc undergoing therapy with either bosentan (n = 11) or iloprost (n = 16). Standardized photodocumentations of all patients (n = 27) were obtained at a time point ten months after therapy initiation and analyzed. A subgroup of patients (bosentan: n = 6; iloprost: n = 6) was additionally photodocumented prior to therapy initiation, enabling an intraindividual analysis over the course of therapy.
  
  
  RESULTS
  After ten months of therapy patients with SSc receiving bosentan showed a significantly (P = 0.0028) higher frequency of centrofacial TAE (41.6 ± 27.8) as compared to patients with SSc receiving iloprost (14.3 ± 13.1). Detailed subgroup analysis revealed that the frequency of TAE in the bosentan group (n = 6 patients) increased markedly and significantly (P = 0.027) by 44.4 after ten months of therapy (TAE at therapy initiation: 10.8 ± 5.1; TAE after ten months of therapy: 55.2 ± 29.8), whereas an only minor increase of 1.9 was observed in the iloprost group (n = 6 patients; TAE at therapy initiation: 18.3 ± 14.5; TAE after ten months of therapy: 20.2 ± 15.5), yet without reaching statistical significance (P = 0.420).
  
  
  CONCLUSIONS
  The use of bosentan may be associated with an increased frequency of TAE in patients with SSc. Patients should be informed about this potential adverse effect prior to therapy. Treatment options may include camouflage or laser therapy.
authors.forename: Sonja
authors.initials: S
authors.lastname: Hetzer
authors.forename: Bettina Alexandra
authors.initials: BA
authors.lastname: Buhren
authors.forename: Holger
authors.initials: H
authors.lastname: Schrumpf
authors.forename: Edwin
authors.initials: E
authors.lastname: Bölke
authors.forename: Stephan
authors.initials: S
authors.lastname: Meller
authors.forename: Kai
authors.initials: K
authors.lastname: Kammers
authors.forename: Peter Arne
authors.initials: PA
authors.lastname: Gerber
authors.affiliation: Department of Dermatology, Medical Faculty, University of Duesseldorf, Moorenstrasse 5, D-40225 Duesseldorf, Germany. bernhard.homey@uni-duesseldorf.de.
authors.forename: Bernhard
authors.initials: B
authors.lastname: Homey
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000077300:Bosentan; D005145:Face / Q000098:blood supply; D005260:Female; D006801:Humans; D016285:Iloprost / Q000009:adverse effects*; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000188:drug therapy*; D013449:Sulfonamides / Q000009:adverse effects*; D013684:Telangiectasis / Q000139:chemically induced*
chemical_list: D013449:Sulfonamides; D016285:Iloprost; D000077300:Bosentan