Ahmadi-Simab K, Köhler A, Gross W L
Department of Rheumatology, University Hospital of Schleswig-Holstein, Campus Lübeck and Rheumaklinik Bad Bramstedt, Germany.
Clin Exp Rheumatol. 2007 Sep-Oct;25(5):760-2.
We report here the case of a woman with diffuse cutaneous systemic sclerosis with pulmonary involvement and severe (WHO functional class III) pulmonary arterial hypertension (PAH) and recurrent cardiac decompensation. The simultaneous presence of primary biliary cirrhosis with markedly elevated transaminase levels constituted a contraindication for bosentan, which would otherwise have been the first-line treatment for PAH. The patient was therefore treated with inhaled iloprost.
Once inhaled iloprost therapy had been started, we promptly noted a definite and sustained improvement in physical exercise capacity and normalisation of haemodynamic variables. In cases where bosentan is contraindicated, inhaled iloprost is an effective alternative for the treatment of severe PAH.
我们在此报告一例患有弥漫性皮肤系统性硬化症且伴有肺部受累、重度（世界卫生组织功能分级III级）肺动脉高压（PAH）及反复心脏失代偿的女性病例。原发性胆汁性肝硬化伴转氨酶水平显著升高同时存在，构成了波生坦治疗的禁忌证，否则波生坦本应是PAH的一线治疗药物。因此，该患者接受了吸入伊洛前列素治疗。
一旦开始吸入伊洛前列素治疗，我们立即注意到患者的身体运动能力有明确且持续的改善，血流动力学变量也恢复正常。在波生坦禁忌的情况下，吸入伊洛前列素是治疗重度PAH的有效替代药物。
