Pulmonary hypertension in systemic sclerosis: An analysis of 17 patients

E. T. Koh, P. Lee, D. D. Gladman, M. Abu-Shakra

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288 Scopus citations


A retrospective chart review was carried out on 344 patients with systemic sclerosis (SSc) followed prospectively for the occurrence of pulmonary hypertension (PHT). Seventeen patients (4.9%) were found to have PHT. Eight patients had isolated PHT, while in nine PHT was associated with restrictive lung disease (RLD). The subset with RLD developed PHT earlier, but had longer survival than patients with isolated PHT. Patients with limited scleroderma tend to develop isolated PHT, while in those with diffuse disease PHT is associated with RLD. Irrespective of disease type, PHT in SSc has an extremely poor prognosis with a median survival of 12 months following diagnosis.

Original languageEnglish
Pages (from-to)989-993
Number of pages5
JournalBritish Journal of Rheumatology
Issue number10
StatePublished - 30 Oct 1996
Externally publishedYes


  • Pulmonary hypertension
  • Scleroderma
  • Systemic sclerosis

ASJC Scopus subject areas

  • Rheumatology
  • Neuroscience (all)


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