Valentini G, Paone C, La Montagna G, Chiarolanza I, Menegozzo M, Colutta E, Ruocco L
The Rheumatology Health Unit, Second University of Naples, Italy.
Scand J Rheumatol. 2006 Jan-Feb;35(1):35-8. doi: 10.1080/03009740510026896.
To investigate the efficacy of a treatment with low-dose intravenous cyclophosphamide (CYC) and low-dose prednisone in early diffuse cutaneous systemic sclerosis (dcSSc).
Patients with dcSSc and a disease duration <24 months consecutively admitted to a tertiary centre underwent a prospective 1-year study. They were treated with i.v. CYC 500 mg/pulses, 10 mg prednisone equivalent, and supportive therapy. Modified Rodnan skin score (mRss), Health Assessment Questionnaire-Disability Index (HAQ-DI), forced vital capacity (FVC), and diffusing lung capacity for CO (DLCO) were assessed as outcome measures. In addition, the nine Medsger severity scale scores were evaluated.
mRss and DLCO significantly improved at both 6 (p = 0.002 and 0.012, respectively) and 12 months (p = 0.002 and 0.003, respectively). HAQ-DI showed a nearly significant reduction at 12 months (p = 0.06). Medsger's severity scores also improved for general condition (p = 0.001), peripheral vascular (p = 0.05), skin (p = 0.02), joint/tendon (p = 0.001), muscle (p = 0.05), and lung (p = 0.02). No treatment interruption was needed.
This preliminary study suggests a role for low-dose i.v. CYC in the treatment of early dcSSc. Controlled studies are warranted.
探讨低剂量静脉注射环磷酰胺(CYC)和低剂量泼尼松治疗早期弥漫性皮肤系统性硬化症(dcSSc)的疗效。
连续入住三级中心、病程<24个月的dcSSc患者进行了一项为期1年的前瞻性研究。他们接受静脉注射CYC 500mg/脉冲、10mg等效泼尼松及支持治疗。评估改良罗德南皮肤评分(mRss)、健康评估问卷残疾指数(HAQ-DI)、用力肺活量(FVC)和一氧化碳弥散量(DLCO)作为疗效指标。此外,还评估了九分梅兹格严重程度量表评分。
mRss和DLCO在6个月(分别为p = 0.002和0.012)和12个月(分别为p = 0.002和0.003)时均显著改善。HAQ-DI在12个月时显示出接近显著的降低(p = 0.06)。梅兹格严重程度评分在一般状况(p = 0.001)、外周血管(p = 0.05)、皮肤(p = 0.02)、关节/肌腱(p = 0.001)、肌肉(p = 0.05)和肺部(p = 0.02)方面也有所改善。无需中断治疗。
这项初步研究表明低剂量静脉注射CYC在早期dcSSc治疗中具有一定作用。有必要进行对照研究。