Pulmonary hypertension with limited cutaneous scleroderma (CREST syndrome).
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.
Berends
J C
JC
Isala Klinieken Locatie Weezenlanden, P.O. Box 10500, 8000 GM, Zwolle, Netherlands. j.c.berends@int.azg.nl
Dompeling
E C
EC
van der Star
J G
JG
Hoorntje
J C
JC
D000792:Angiography; D017675:CREST Syndrome / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D018450:Disease Progression; D004452:Echocardiography; D017809:Fatal Outcome; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests; D014057:Tomography, X-Ray Computed