[Primary pulmonary hypertension: a rare but often fatal complication of Crest syndrome]

Rev Med Interne. 1997;18(2):109-13. doi: 10.1016/s0248-8663(97)84675-9.
[Article in French]

Abstract

In a retrospective study of 194 systemic sclerosis, we selected 37 Crest syndrome patients. Among these 37 patients, five presented a primary pulmonary hypertension. For three of these patients, the evolution of this primary pulmonary hypertension was rapidly fatal, one died of another cause. One died without certitude that death is imputable to HTAP. Diagnosis and management of the evolution of primary pulmonary hypertension may be easily assessed by doppler-echocardiography.

Publication types

  • Case Reports
  • English Abstract
  • Review

MeSH terms

  • Aged
  • CREST Syndrome / complications*
  • CREST Syndrome / physiopathology
  • CREST Syndrome / therapy
  • Fatal Outcome
  • Female
  • Humans
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy
  • Middle Aged
  • Retrospective Studies