| 摘要: |
| ［摘要］ 目的 分析环磷酰胺（CYC）治疗抗合成酶综合征相关间质性肺疾病（ASS-ILD）的临床疗效及影响因素。方法 回顾性分析2020年1月至2024年5月南京医科大学第三附属医院及苏州大学附属第三医院收治的111例ASS-ILD患者的临床资料。根据初始治疗方案将患者分为CYC组（49例）和非CYC组（62例）。根据血清抗体状态将CYC组患者分为抗Jo-1抗体阴性组和抗Jo-1抗体阳性组。根据基线（治疗前）肺功能情况将CYC组患者分为用力肺活量占预计值百分比（FVC%pred）<70%组和FVC%pred≥70%组。比较各组基线资料、治疗前和治疗后6～12个月肺功能变化及1年随访资料。结果 与非CYC组比较，CYC组抗Jo-1抗体阳性率、肌无力症状比例及白细胞计数（WBC）、血红蛋白（Hb）、肌酸激酶同工酶（CK-MB）水平较高（P<0.05）。随访期间，CYC组FVC%pred改善幅度大于非CYC组，差异有统计学意义（P<0.05），且CYC组未出现FVC%pred较基线下降≥10%的患者，而非CYC组中FVC%pred较基线下降≥10%的患者比例为14.52%。与非CYC组比较，CYC组因疗效好而减量/停药的比例较高，1年后泼尼松日平均剂量较低，差异有统计学意义（P<0.05）。与CYC治疗前比较，抗Jo-1抗体阴性组与抗Jo-1抗体阳性组治疗后FVC%pred显著升高（P<0.05）；两组治疗前后一氧化碳弥散量占预计值百分比（DLCO%pred）比较差异无统计学意义（P>0.05）。CYC治疗后，FVC%pred<70%组FVC%pred改善幅度大于FVC%pred≥70%组，差异有统计学意义（P<0.05）。结论 CYC治疗ASS-ILD疗效显著，尤其适用于进展型患者及重症患者，可作为此类患者的首选治疗方案。 |
| 关键词: 环磷酰胺 抗合成酶综合征 间质性肺疾病 肺功能 |
| DOI：10.3969/j.issn.1674-3806.2026.03.12 |
| 分类号: |
| 基金项目:常州市卫生健康委科技项目（编号：ZD202344） |
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| Analysis on clinical efficacy and influencing factors of cyclophosphamide in treatment of anti-synthetase syndrome-associated interstitial lung disease |
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XUE Dingying1, LI Mingting1, WU Min2, GENG Yaqin1, ZHOU Lei1, ZHANG Yu1, QIAN Hao1, GAO Bo1
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1.Department of Rheumatology and Immunology, the Third Affiliated Hospital of Nanjing Medical University(Changzhou NO.2 People′s Hospital), Changzhou 213000, China; 2.Department of Rheumatology and Immunology, the Third Affiliated Hospital of Soochow University(the First People′s Hospital of Changzhou), Changzhou 213000, China
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| Abstract: |
| ［Abstract］ Objective To analyze the clinical efficacy and influencing factors of cyclophosphamide(CYC) in treatment of anti-synthetase syndrome-associated interstitial lung disease(ASS-ILD). Methods A retrospective analysis was conducted on the clinical data of 111 patients with ASS-ILD who were admitted to the Third Affiliated Hospital of Nanjing Medical University and the Third Affiliated Hospital of Soochow University from January 2020 to May 2024. According to different initial therapeutic regimens, the patients were divided into CYC group(49 patients) and non-CYC group(62 patients). The patients in the CYC group were divided into anti-histidyl transfer RNA(tRNA) synthetase(Jo-1) antibody negative group and anti-Jo-1 antibody positive group according to their different serum antibody states. The patients in the CYC group were divided into forced vital capacity percent predicted(FVC%pred)<70% group and FVC%pred≥70% group according to their different baselines of lung function(before treatment). The baseline data, the changes in lung function before treatment and 6 to 12 months after treatment, and the 1-year follow-up data were compared between two groups. Results Compared with the non-CYC group, the CYC group had the significantly elevated anti-Jo-1 antibody positive rate, proportion of myasthenia symptoms and white blood cell count(WBC), hemoglobin(Hb), and creatinine kinase-myocardial band(CK-MB) levels (P<0.05). During the follow-up period, the improvement extent of FVC%pred in the CYC group was greater than that in the non-CYC group, with statistically significant difference between the two groups(P<0.05). In the CYC group, there were no patients with a decrease in FVC%pred≥10% compared to the baseline data, while the proportion of the patients with a decrease in FVC%pred≥10% compared to the baseline data in the non-CYC group was 14.52%. Compared with the non-CYC group, the CYC group had high proportion of drug dosage reduction or withdrawal due to better therapeutic effect, and 1 year after treatment, the average daily dose of prednisone in the CYC group was low, and the differences were statistically significant(P<0.05). Compared with those before CYC treatment, the levels of FVC%pred in the anti-Jo-1 antibody negative group and the anti-Jo-1 antibody positive group were significantly elevated after CYC treatment(P<0.05). There were no statistically significant differences in the levels of diffusing capacity of the lung for carbon monoxide percent predicted(DLCO%pred) between the two groups before and after treatment(P>0.05). After CYC treatment, the improvement extent of FVC%pred in the FVC%pred<70% group was greater than that in the FVC%pred≥70% group, and the difference was statistically significant(P<0.05). Conclusion CYC has a significantly therapeutic effect on ASS-ILD, especially for progressive patients and severe patients, and can be used as the preferred treatment regimen for these patients. |
| Key words: Cyclophosphamide(CYC) Anti-synthetase syndrome(ASS) Interstitial lung disease Lung function |
