The recurrence of digital ulcers in patients with systemic sclerosis after discontinuation of oral treprostinil

Ami A. Shah, Elena Schiopu, Soumya Chatterjee, Mary Ellen Csuka, Tracy Frech, Avram Goldberg, Robert Spiera, Stanford L. Peng, Ryan J. McBride, Jody M. Cleveland, Virginia Steen

Research output: Contribution to journalArticlepeer-review

12 Scopus citations


Objective. Prior studies investigating the efficacy of oral treprostinil to treat digital ulcers (DU) in systemic sclerosis (SSc)-associated Raynaud phenomenon have yielded conflicting results. In this investigation, we examined whether DU burden increased after patients withdrew from oral treprostinil that was administered during an open-label extension study. Methods. A multicenter, retrospective study was conducted to determine DU burden in the year after withdrawal from oral treprostinil. DU burden 3-6 months (Time A) and > 6-12 months (Time B) after drug withdrawal was compared with DU burden at baseline, defined as the last day receiving drug in the open-label extension study, by a paired Student t test. Changes in DU burden while receiving drug in the open-label study were compared with changes in DU burden at Time B by a paired Student t test. Results. Fifty-one patients from 9 clinical sites were included for analysis. DU burden increased significantly from baseline (mean 0.47) to Time A (mean 2.1, p = 0.002, n = 23) and Time B (mean 1.45, p = 0.013, n = 30). Total DU burden decreased during oral treprostinil exposure (mean change -0.6) and then increased by Time B (mean change 1.05, p = 0.0027 for comparison, n = 30). In the year after drug withdrawal, many patients required vasodilator therapy and pain medications. Three patients were hospitalized for complications from DU, and 4 patients required surgery for DU. Conclusion. Total DU burden increased significantly after discontinuation of oral treprostinil. These data provide supportive evidence of a beneficial effect of oral treprostinil for the vascular complications of SSc and suggest that further study is warranted.

Original languageEnglish (US)
Pages (from-to)1665-1671
Number of pages7
JournalJournal of Rheumatology
Issue number9
StatePublished - Sep 2016
Externally publishedYes


  • Digital ulcers
  • Raynaud phenomenon
  • Systemic sclerosis
  • Treprostinil diolamine

ASJC Scopus subject areas

  • Rheumatology
  • Immunology and Allergy
  • Immunology


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