Airò P, Danieli E, Rossi M, Frassi M, Cavazzana I, Scarsi M, Grottolo A, Franceschini F, Zambruni A
Rheumatology, Allergology and Clinical Immunology, Spedali Civili, Brescia, Italy.
Clin Exp Rheumatol. 2007 Mar-Apr;25(2):293-6.
Cyclophosphamide (CYC) is generally considered the most promising agent available today for systemic sclerosis (SSc)-related interstitial lung disease (ILD). However, the optimal dosage and length of treatment are still undetermined. Our objective was to evaluate the effect of an 18-month long protocol with intravenous (iv) CYC.
In a single-centre, prospective, observational study, 13 patients with SSc and active alveolitis were given 8 iv pulses in a 6-months period (CYC 750 mg + 6-methylprednisolone 125 mg every three weeks), as an induction therapy. Patients received maintenance therapy with further cycles at 4 (3 pulses), 6 (3 pulses) and 9 weeks (3 pulses) interval. Total CYC dosage was 12.75 g in an 18-month period. End-points were modifications of lung function test (LFT).
During the first 6 months of treatment with CYC an increase in Forced Vital Capacity (FVC; p = 0.005) and in diffusion lung capacity for carbon monoxide (DLCO; p = 0.10) was observed; during the maintenance therapy, there was a stabilization in FVC and a mild, non significant decline in DLCO. Treatment was well tolerated.
iv CYC can induce an initial improvement in LFT (particularly, in FVC) in the first six months, but no further improvement was observed during the maintenance phase.
环磷酰胺（CYC）通常被认为是目前治疗系统性硬化症（SSc）相关间质性肺疾病（ILD）最有前景的药物。然而，最佳剂量和治疗时长仍未确定。我们的目的是评估静脉注射CYC为期18个月方案的效果。
在一项单中心、前瞻性观察研究中，13例患有SSc且有活动性肺泡炎的患者在6个月内接受8次静脉脉冲治疗（每三周给予CYC 750 mg + 6-甲基泼尼松龙125 mg），作为诱导治疗。患者在4周（3次脉冲）、6周（3次脉冲）和9周（3次脉冲）间隔时接受进一步疗程的维持治疗。在18个月期间CYC总剂量为12.75 g。终点指标为肺功能测试（LFT）的变化。
在使用CYC治疗的前6个月，观察到用力肺活量（FVC；p = 0.005）和一氧化碳弥散肺容量（DLCO；p = 0.10）增加；在维持治疗期间，FVC稳定，DLCO有轻度、无显著意义的下降。治疗耐受性良好。
静脉注射CYC可在最初6个月使LFT（特别是FVC）初步改善，但在维持阶段未观察到进一步改善。
