Airò P, Rossi M, Scarsi M, Danieli E, Grottolo A, Zambruni A
Rheumatology and Clinical Immunology Clinic, Spedali Civili, Brescia, Italy.
Clin Exp Rheumatol. 2007 Sep-Oct;25(5):722-7.
To evaluate the role of iloprost, a derivative of prostacyclin, as a possible disease-modifying agent for systemic sclerosis (SSc).
Fifty-six consecutive SSc patients treated for a median period of 4 years with cyclic infusions of iloprost for severe Raynaud's phenomenon and ischemic ulcers were compared with 56 control patients matched for age, sex, disease subset and duration. Control patients were also similar to the iloprost group with regard to autoantibody status, the presence of major disease-related organ manifestations at baseline, and the use of other treatments. The evolution of lung function test results, the frequency of major disease-specific complications and the survival of the cohorts were the objects of this analysis.
No significant difference was observed between the two groups with regard to changes in lung function tests over time, or the number of patients who presented with the onset of active interstitial lung disease, pulmonary arterial hypertension or scleroderma renal crisis. Survival did not differ between the two groups.
The evolution of lung function test results, the frequency of major disease-specific complications, and survival did not differ significantly between SSc patients treated with cyclic iloprost and a group of patients matched for sex, age, and disease subset and duration. However, no cases of severe pulmonary arterial hypertension were observed in the patients treated with iloprost, suggesting that studies focusing on the possible preventive action of iloprost on the progression of SSc- associated mild pulmonary arterial hypertension would be warranted.
评估前列环素衍生物伊洛前列素作为系统性硬化症（SSc）潜在疾病改善药物的作用。
将56例因严重雷诺现象和缺血性溃疡接受伊洛前列素周期性输注治疗、中位治疗时间为4年的连续性SSc患者，与56例在年龄、性别、疾病亚型和病程方面相匹配的对照患者进行比较。对照患者在自身抗体状态、基线时主要疾病相关器官表现的存在情况以及其他治疗的使用方面也与伊洛前列素组相似。本分析的对象是肺功能测试结果的演变、主要疾病特异性并发症的发生频率以及队列的生存率。
两组在肺功能测试随时间的变化、出现活动性间质性肺病、肺动脉高压或硬皮病肾危象发作的患者数量方面均未观察到显著差异。两组的生存率无差异。
接受周期性伊洛前列素治疗的SSc患者与在性别、年龄、疾病亚型和病程方面相匹配的一组患者相比，肺功能测试结果的演变、主要疾病特异性并发症的发生频率和生存率均无显著差异。然而，在接受伊洛前列素治疗的患者中未观察到严重肺动脉高压病例，这表明有必要开展侧重于伊洛前列素对SSc相关轻度肺动脉高压进展可能预防作用的研究。
