Abstract
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 language | English |
---|---|
Pages (from-to) | 989-993 |
Number of pages | 5 |
Journal | British Journal of Rheumatology |
Volume | 35 |
Issue number | 10 |
State | Published - 30 Oct 1996 |
Externally published | Yes |
Keywords
- Pulmonary hypertension
- Scleroderma
- Systemic sclerosis
ASJC Scopus subject areas
- Rheumatology
- General Neuroscience