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系统性硬化病肺间质病变临床分析

Clinical analysis of interstitial lung disease in systemic sclerosis

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目的 回顾性分析202例系统性硬化病(SSc)合并肺间质病变(ILD)患者的疾病特点,研究不同分型SSc-ILD的特征及其与血管病变及抗体的相关性,加强对SSc-ILD的疾病认识.方法 收集2021年5月至2022年9月在上海市中西医结合医院住院的249例SSc患者的病例资料,分别通过SSc-ILD与SSc非ILD 2组间比较以及SSc-ILD组内不同分型之间的比较,分析SSc-ILD的疾病特点,及其与血管病变、自身抗体之间的相关性.统计分析方法根据数据类型,分别采用配对t检验、Wilcoxon符号秩和检验或x2检验.结果 本研究中202例SSc-ILD患者中女性患者183例(90.6%),47例SSc非ILD患者中女性患者36例(76.6%),女性SSc患者出现ILD的发生率明显高于男性(x2=7.05,P=0.008).胸部高分辨率CT(HRCT)发现磨玻璃影最常见(90.1%);小叶内间隔增厚、网状影的发生率均高达70.0%以上;其次是小叶间隔增厚、支气管牵拉,发生率均高于50.0%.普遍型间质性肺炎(UIP)组肺纤维化积分[23.77(10.69,36.54)]高于非特异性间质性肺炎(NSIP)组[12.63(3.44,17.89),Z=5.19,P<0.001];NSIP(纤维化型)(f-NSIP)组肺纤维化积分[18.55(6.52,26.06)]高于 NSIP(细胞型)(c-NSIP)组[5.27(1.15,7.55),Z=7.59,P<0.001];肺纤维化积分与SSc发病年龄呈正相关(r=0.02,P=0.021).UIP患者病程中伴发毛细血管扩张(37.5%与 19.9%,x2=2.60,P=0.009)、皮肤溃疡(48.2%与 31.5%,x2=2.21,P=0.027)及指骨吸收(51.8%与28.1%,x2--3.17,P=0.002)的发生率高于NSIP;f-NSIP组病程中出现毛细血管扩张(27.2%与10.8%,x2=2.26,P=0.024)与指骨吸收(35.8%与18.5%,x2=2.32,P=0.021)的发生率高于c-NSIP组.SSc-ILD患者的抗Scl-70抗体阳性率(76.2%与23.4%)明显高于SSc非ILD组患者(x2=47.61,P<0.001);而SSc非ILD组患者的抗着丝点抗体阳性率(31.9%与11.4%)明显高于SSc-ILD组患者(x2=12.42,P=0.001)o SSc-ILD组患者的抗SSB抗体阳性率(9.9%与0)具有显著性差异(P=0.025).结论 抗Scl-70抗体阳性与SSc-ILD的发生相关,抗SSB抗体阳性可能是SSc患者出现ILD的危险因素.
Objective Retrospective analyzed the characteristics of 202 patients with interstitial lung disease(ILD)in systemic sclerosis(SSc),studied the relationship between their characteristics in different types of SSc-ILD and vascular disease and antibody,in order to increase the understanding of SSc-ILD.Methods A total of 249 inpatients with SSc in our hospital from May 2021 to September 2022 were invloved.By comparing SSC-ILD with SSc-non-ILD groups and different types in SSc-ILD groups,to analyze the relationship between their characteristics of SSc-ILD and vascular disease and auto-antibody.According to the data type,the paired sample t test,Wilcoxon signed rank sum test,ANOVA analysis of variance or Chi-square test were used for statistical analysis.The statistical analysis software to analyze the relationship between their characteristics of SSc-ILD and vascular disease and auto-antibody.According to the data type,the paired sample t test,Wilcoxon signed rank sum test,or Chi-square test were used for statistical analysis.Results In this study,among 202 patients with SSC-ILD,183 were female(90.6%),and among 47 patients with SSc-non-ILD,36 were female(76.6%).The incidence of ILD in female SSc patients was significantly higher than that in male patients(x2=7.05,P=0.008).The chest high-resolution CT(HRCT)showed that ground glass was the most common presentation(90.1%).The incidence of interlobular septum thickening and reticular shadow were more than 70.0%.The incidence of interlobular thickening and bronchial distraction was higher than 50.0%.The pulmonary fibrosis score[23.77(10.69,36.54)]in the usual interstitial pneumonia(UIP)group was higher than that in the nonspecific interstitial pneumonia(NSIP)group[12.63(3.44,17.89)](Z=5.19,P<0.001).The pulmonary fibrosis score[18.55(6.52,26.06)]in the nonspecific interstitial pneumonia(the fibrotic type)(f-NSIP)group was higher than that in the nonspecific interstitial pneumonia(cellular type)(c-NSIP)group[5.27(1.15,7.55),Z=7.59,P<0.001].The score of pulmonary fibrosis was positively correlated with the age of onset of SSc(r=0.02,P=0.021).The incidence of telangiectasia(37.5%vs.19.9%,x2=2.60,P=0.009),skin ulcer(48.2%vs.31.5%,x2=2.21,P=0.027)and phalangeal resorption(51.8%vs.28.1%,x2=3.17,P=0.002)in UIP patients was higher than that of NSIP.The incidence of telangiectasia(27.2%vs.10.8%,x2=2.26,P=0.024)and phalangeal resorptive(35.8%vs.18.5%,x2=2.32,P=0.021)in the f-NSIP group was higher than that in the c-NSIP group.The positive rate of anti-SCL-70 antibody in SSC-ILD patients(76.2%vs.23.4%)was significantly higher than that in SSc non-ILD patients(x2=47.61,P<0.001).The positive rate of anti-centromere antibody in SSc non-ILD group(31.9%vs.11.4%)was significantly higher than that in SSC-ILD group(x2=12.42,P=0.001).There was a significant difference in the positive rate of anti-SSB antibody in SSc-ILD group(9.9%vs.0,P=0.025).Conclusion Positive anti-SCL-70 antibody is directly related to the occurrence of SSC-ILD,and positive anti-SSB antibody may be a risk factor for ILD in patients with SSc.

Sclerosis,systemicLunginterstitial diseaseUsual interstitial pneumoniaNonspecific interstitial pneumoniaAuto-antibody

王蕾、郗艳、孔祥贞、屠文震、舒政、陈园园、孙大燕、何东仪

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上海中医药大学附属上海市中西医结合医院风湿病科,上海 200082

上海中医药大学附属上海市中西医结合医院放射科,上海 200082

复旦大学生命科学院,上海 200438

首都医科大学附属北京儿童医院新生儿外科,北京 100045

上海中医药大学附属光华医院关节内科,上海 200052

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硬化病,系统性 肺炎纤维化 普遍型间质性肺炎 非特异性间质性肺炎 自身抗体

上海市虹口区卫生健康委医学科研项目(2023-2025)上海中医药大学附属医院临床研究型骨干人才培养计划

2023LCRC22

2024

中华风湿病学杂志
中华医学会

中华风湿病学杂志

CSTPCD
影响因子:0.651
ISSN:1007-7480
年,卷(期):2024.28(4)
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