Su Tien-I Karleen, Khanna Dinesh, Furst Daniel E, Danovitch Gabriel, Burger Christina, Maranian Paul, Clements Philip J
University of Southern California, Los Angeles, CA 90095-1670, USA.
Arthritis Rheum. 2009 Dec;60(12):3821-30. doi: 10.1002/art.24986.
To assess the safety and efficacy of rapamycin in the treatment of diffuse systemic sclerosis (SSc; scleroderma).
Eighteen patients with diffuse SSc of <or=5 years duration were randomized to receive rapamycin or methotrexate (MTX) in a single-blind, 48-week study. Abnormalities in clinical and laboratory parameters were compared between the 2 treatment groups. The potential efficacy of the study drugs was evaluated by comparing results of the baseline and 48-week assessments, including the modified Rodnan skin thickness score (MRSS) and the Health Assessment Questionnaire disability index.
The baseline characteristics of the patients were similar in both groups (n=9 in each). One patient in the rapamycin group who never received the study drug was excluded from the analysis. Three patients in each group withdrew from the study; 2 of the withdrawals were treatment-related (severe hypertriglyceridemia associated with rapamycin, and pancytopenia associated with MTX), and 4 were SSc-related. Hypertriglyceridemia was the most notable side effect associated with rapamycin, but it was generally well tolerated and treatable. The incidence and severity of other adverse drug reactions were comparable between the 2 groups. Within each group, the MRSS improved significantly from baseline. In the rapamycin group, the patient's global assessment showed a significant improvement from baseline, while forced vital capacity values declined from baseline. The disease activity scores at 48 weeks and the changes in these scores from baseline were not significantly different between the 2 groups.
Rapamycin has a reasonable safety profile in a select group of patients with scleroderma. Larger trials are needed to assess the efficacy of rapamycin in patients with early diffuse SSc.
评估雷帕霉素治疗弥漫性系统性硬化症(SSc;硬皮病)的安全性和有效性。
在一项为期48周的单盲研究中,将18例病程≤5年的弥漫性SSc患者随机分为雷帕霉素组或甲氨蝶呤(MTX)组。比较两组临床和实验室参数的异常情况。通过比较基线和48周评估结果,包括改良Rodnan皮肤厚度评分(MRSS)和健康评估问卷残疾指数,评估研究药物的潜在疗效。
两组患者的基线特征相似(每组n = 9)。雷帕霉素组中1例从未接受研究药物的患者被排除在分析之外。每组有3例患者退出研究;其中2例退出与治疗相关(雷帕霉素相关的严重高甘油三酯血症和MTX相关的全血细胞减少),4例与SSc相关。高甘油三酯血症是与雷帕霉素相关的最显著副作用,但通常耐受性良好且可治疗。两组其他药物不良反应的发生率和严重程度相当。每组内,MRSS较基线均有显著改善。在雷帕霉素组中,患者的整体评估较基线有显著改善,而用力肺活量值较基线下降。两组在48周时的疾病活动评分及这些评分较基线的变化无显著差异。
雷帕霉素在特定的硬皮病患者群体中具有合理的安全性。需要进行更大规模的试验来评估雷帕霉素在早期弥漫性SSc患者中的疗效。