Kowal-Bielecka O, Delcroix M, Vonk-Noordegraaf A, Hoeper M M, Naeije R
Department of Rheumatology and Internal Medicine, Medical University of Bialystok, Bialystok, Poland.
Rheumatology (Oxford). 2008 Oct;47 Suppl 5:v39-41. doi: 10.1093/rheumatology/ken308.
SSc is complicated in approximately 10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea-fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of approximately 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor antagonists and phosphodiesterase-5 inhibitors have been shown to be efficacious in PAH, with persistent long-term benefit and approximate doubling of survival rate, and these encouraging results appear transposable to the SSc-PAH subcategory. However, PAH as well as SSc-PAH remain incurable, with insufficient functional improvement in many patients. More progress is needed, and this will require more effective drugs and adapted outcome measures.
约10%的系统性硬化症（SSc）患者会并发肺动脉高压（PAH），这是一种由肺血管阻力增加引起的罕见的呼吸困难-疲劳综合征。SSc相关PAH的预后特别差，在没有针对肺循环的治疗的情况下，2年的估计生存率约为50%。前列环素、内皮素受体拮抗剂和磷酸二酯酶-5抑制剂已被证明对PAH有效，具有持续的长期益处且生存率大约提高一倍，这些令人鼓舞的结果似乎也适用于SSc相关PAH亚类。然而，PAH以及SSc相关PAH仍然无法治愈，许多患者的功能改善不足。还需要取得更多进展，这将需要更有效的药物和合适的疗效评估指标。
