Berends J C, Dompeling E C, van der Star J G, Hoorntje J C
Isala Klinieken Locatie Weezenlanden, P.O. Box 10500, 8000 GM, Zwolle, Netherlands.
Neth J Med. 2000 Dec;57(6):229-32.
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.
本文描述了一名患有与局限性皮肤型硬皮病（也称为CREST综合征）相关的典型肺动脉高压表现的患者。该患者接受了钙拮抗剂、口服抗凝治疗，并且由于有肺部实质炎症的证据，还接受了小剂量泼尼松和环磷酰胺治疗。这种治疗使弥散能力和运动耐力最初得到改善，然而，诊断后1年患者死于进行性肺动脉高压。
