Tzelepis G E, Plastiras S C, Karadimitrakis S P, Vlachoyiannopoulos P G
Department of Pathophysiology, National University of Athens School of Medicine, Athens, Greece.
Clin Exp Rheumatol. 2007 Sep-Oct;25(5):734-9.
In patients with scleroderma-related interstitial lung disease (ILD), improvements of pulmonary function have been reported after treatment with cyclophosphamide (CYC) alone or CYC and high-dose steroids. The study objective was to identify therapeutic regimen that alone or in combination with laboratory or clinical characteristics were associated with pulmonary function improvement in these patients.
Scleroderma patients with ILD and serial pulmonary function measurements were retrospectively analyzed. We recorded forced vital capacity (FVC, % predicted), diffusion capacity (DLCO, % predicted), type of therapy, and various clinical and laboratory parameters. Treatment with IV CYC was recorded as cumulative dose (grams) and treatment with steroids as high or low dose; outcome was defined as a sustained increase in FVC (% predicted) >or= 10 points.
Of the 59 patients who were included in the study, 29 (49 %) patients received IV CYC (cumulative dose 13.9 +/-6.2, range 5.2-26.2 gr) for 3.3 +/- 2.4 years (range 5-60 months). Eighteen out of 59 (30 %) patients received high-dose prednisolone and 41 (70 %) received low-dose prednisolone. In an ordinal logistic model, patients receiving > 12 gr of CYC were 6 times more likely to improve FVC than to decrease or maintain FVC, compared to those who did not receive CYC (p = 0.02). In multivariate analysis, the effect of high dosage CYC on FVC persisted (OR 10.82, p = 0.02). Steroid dosage (high or low) was not associated with FVC improvement (p < 0.05).
In patients with scleroderma and ILD, treatment with CYC is the only variable that is independently associated with pulmonary function improvement and that prolonged (> 1 year) CYC therapy increases the probability of pulmonary function improvement more than shorter CYC courses.
在硬皮病相关间质性肺疾病（ILD）患者中，据报道单独使用环磷酰胺（CYC）或联合使用CYC与大剂量类固醇治疗后肺功能有所改善。本研究的目的是确定单独使用或与实验室或临床特征联合使用时，与这些患者肺功能改善相关的治疗方案。
对患有ILD且有系列肺功能测量数据的硬皮病患者进行回顾性分析。我们记录了用力肺活量（FVC，预测值百分比）、弥散功能（DLCO，预测值百分比）、治疗类型以及各种临床和实验室参数。静脉注射CYC的治疗记录为累积剂量（克），类固醇治疗记录为高剂量或低剂量；结局定义为FVC（预测值百分比）持续增加≥10分。
纳入研究的59例患者中，29例（49%）接受静脉注射CYC（累积剂量13.9±6.2，范围5.2 - 26.2克），治疗3.3±2.4年（范围5 - 60个月）。59例患者中有18例（30%）接受高剂量泼尼松龙，41例（70%）接受低剂量泼尼松龙。在有序逻辑模型中，与未接受CYC的患者相比，接受>12克CYC的患者FVC改善的可能性是FVC降低或维持的6倍（p = 0.02）。在多变量分析中，高剂量CYC对FVC的影响持续存在（OR 10.82，p = 0.02）。类固醇剂量（高或低）与FVC改善无关（p < 0.05）。
在硬皮病和ILD患者中，CYC治疗是唯一与肺功能改善独立相关的变量，且延长（>1年）CYC治疗比短期CYC疗程更能增加肺功能改善的可能性。
