title: Low-dose combination therapy of severe digital ulcers in diffuse progressive systemic sclerosis with the endothelin-1 receptor antagonist bosentan and the phosphodiesterase V inhibitor sildenafil.
abstract: Digital ulcers in progressive systemic sclerosis (PSS) are often refractory to therapy. A frequently chronic aggressive course can lead to the loss of acral limbs involved. A 73-year-old woman developed a dramatic worsening of her ulcerations despite maximum conventional therapy. Switching therapy to bosentan and sildenafil, both in low-dose regimens, resulted for the first time in ten years in a complete healing of the ulcers. If substantiated in a series of patients, the additive and perhaps synergistic clinical benefits of combining bosentan and sildenafil may be a valuable option for the treatment of acral ulcers in PSS.
authors.affiliation: Clinic and Policlinic of Dermatology and Venereology, Otto-von-Guericke-University Magdeburg, Leipziger Strasse 44 D-39120 Magdeburg, Germany. Andreas.Ambach@Med.OvGU.de
authors.forename: Andreas
authors.initials: A
authors.lastname: Ambach
authors.forename: Won
authors.initials: W
authors.lastname: Seo
authors.forename: Bernd
authors.initials: B
authors.lastname: Bonnekoh
authors.forename: Harald
authors.initials: H
authors.lastname: Gollnick
mesh_terms: D000368:Aged; D000077300:Bosentan; D004359:Drug Therapy, Combination / Q000379:methods; D065128:Endothelin Receptor Antagonists; D005260:Female; D005385:Fingers; D006229:Hand Dermatoses / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D010726:Phosphodiesterase Inhibitors / Q000008:administration & dosage; D010879:Piperazines / Q000008:administration & dosage*; D011687:Purines / Q000008:administration & dosage; D045743:Scleroderma, Diffuse / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage*; D013450:Sulfones / Q000008:administration & dosage*; D016896:Treatment Outcome; D014456:Ulcer / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology
chemical_list: D065128:Endothelin Receptor Antagonists; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D000068677:Sildenafil Citrate; D000077300:Bosentan