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Updated: May 20, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Survival of patients with pulmonary arterial hypertension, associated with systemic sclerosis]
Aim:
To study survival of patients with a natural course of pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SS).
Material And Methods:
Twenty SD patients with PAH and 71 SS patients free of PAH were followed up for 10 years (1998-2008) when therapy with specific pulmonary vasodilators was not available. Survival curves for both groups were plotted by Kaplan-Mayer method.
Results:
One-, 3- and 5-year survival were 85, 25 and 5%, respectively, in the SS-PAH group and 99, 82 and 77% in the SS group, respectively. The differences were significant (p < 0.001). Survival median in the group SS-PAH was 32 months.
Conclusion:
The above evidence is comparable to the results obtained earlier and demonstrate a fatal outcome of the disease in the absence of therapy with specific pulmonary vasodilators.
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