Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome)
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Updated: Oct 10, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
J C Berends1, E C Dompeling, J G van der Star
1Isala Klinieken Locatie Weezenlanden, P.O. Box 10500, 8000 GM, Zwolle, Netherlands. j.c.berends@int.azg.nl
Abstract:
A patient is described with a typical manifestation of pulmonary hypertension associated with limited cutaneous scleroderma, also known as CREST syndrome. The patient was treated with a calcium antagonist, oral anticoagulation and, because of evidence for parenchymal inflammation of the lung, with low-dose prednisone and cyclophosphamide. This treatment resulted in initial improvement of diffusion capacity and exercise tolerance, however, 1 year after diagnosis the patient died of progressive pulmonary hypertension.
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