目的 探讨复方甘草口服液联合泼尼松治疗特发性肺间质纤维化急性加重期患者的疗效。方法 选取张掖市第二人民医院2020年1月—2023年10月收治的128例特发性肺间质纤维化急性加重期患者,应用随机数字表法分为两组,每组各64例。对照组采取泼尼松治疗,观察组采取复方甘草口服液联合泼尼松治疗。对比其临床疗效,治疗前后血清肺纤维化指标及炎症因子水平,最后对比其不良反应发生率。结果 观察组总有效率93.75%,高于对照组的79.69%（χ²=5.490,P=0.019）;治疗前两组层黏连蛋白（LN）、Ⅲ型前胶原肽（PC-Ⅲ）、透明质酸（HA）对比差异无统计学意义（P>0.05）,治疗后两组患者LN、PC-Ⅲ、HA均降低,观察组[（89.25±7.61）μg/L;（68.33±7.68）mg/L;（81.53±8.54）μg/L]低于对照组[（96.17±8.34）μg/L;（75.68±8.25）mg/L;（95.68±9.25）μg/L],对比差异有统计学意义（t₁=4.903,P₁<0.001;t₂=5.217,P₂<0.001;t₃=8.992,P₃<0.001）;治疗前两组患者肿瘤坏死因子-α（TNF-α）、白细胞介素-4（IL-4）、IL-10、转化生长因子（TGF-β1）对比差异无统计学意义（P>0.05）,治疗后两组TNF-α、IL-4、IL-10、TGF-β1水平降低,观察组[（61.94±8.24）ng/L;（4.43±1.17）ng/mL;（65.49±13.24）g/L;（114.73±13.12）pg/mL]低于对照组[（75.52±9.43）ng/L;（6.31±1.28）ng/mL;（79.27±9.38）g/L;（147.76±15.46）pg/mL],对比差异有统计学意义（t₁=8.675,P₁<0.001;t₂=8.673,P₂<0.001;t₃=6.794,P₃<0.001;t₄=13.032,P₄<0.001）;两组不良反应发生率对比差异无统计学意义（7.81% vs 6.25%,χ²=0.120,P=0.730）。结论 复方甘草口服液联合泼尼松治疗特发性肺间质纤维化急性加重期疗效显著,可辅助延缓患者肺纤维化发展进程,减轻机体炎症反应,安全性较高。

Objective To investigate the efficacy of compound licorice oral liquid combined with prednisone in patients with acute exacerbations of idiopathic pulmonary interstitial fibrosis. Methods A total of 128 patients with acute exacerbation of idiopathic pulmonary interstitial fibrosis admitted to Zhangye Second People's Hospital from January 2020 to October 2023 were divided into two groups by random number table method,with 64 patients in each group．The control group received prednisone treatment,while the observation group received compound licorice oral liquid combined with prednisone treatment．Compare its clinical efficacy,serum pulmonary fibrosis indicators and inflammatory factor levels before and after treatment,and finally compare its incidence of adverse reactions. Results The total response rate in the observation group was 93.75%,which was higher than the 79.69% in the control group（χ²=5.490,P=0.019）．There was no difference between the two groups of LN,PC-III,and HA（P>0.05）,LN,PC-III and HA were decreased in both groups,while the observation group[（89.25±7.61）μg/L;（68.33±7.68）mg/L;（81.53±8.54）μg/L] was lower than the control group[（96.17±8.34）μg/L;（75.68±8.25）mg/L;（95.68±9.25）μg/L],the contrast difference was statistically significant（t₁=4.903,P₁<0.001;t₂=5.217,P₂<0.001;t₃=8.992,P₃<0.001）．There was no difference in TNF-α,IL-4,IL-10,and TGF-β1 between the two groups before treatment（P>0.05）,lower levels of TNF-α,IL-4,IL-10,and TGF-β1 in the two patient groups after treatment,the observation group[（61.94±8.24）ng/L;（4.43±1.17）ng/mL;（65.49±13.24）g/L;（114.73±13.12）pg/mL] was lower than the control group[（75.52±9.43）ng/L;（6.31±1.28）ng/mL;（79.27±9.38）g/L;（147.76±15.46）pg/mL],the contrast difference was statistically significant（t₁=8.675,P₁<0.001;t₂=8.673,P₂<0.001;t₃=6.794,P₃<0.001;t₄=13.032,P₄<0.001）．There was no difference in the incidence of adverse effects between the two groups（7.81% vs 6.25%,χ²=120,P=0.730）. Conclusions The combination of compound licorice oral liquid and prednisone has a significant therapeutic effect on the acute exacerbation of idiopathic pulmonary interstitial fibrosis．It can assist in delaying the progression of pulmonary fibrosis in patients,reducing the body's inflammatory response,and has high safety．

目的 探讨血浆置换联合泼尼松（PDN）联合环磷酰胺（CTX）治疗抗黑色素瘤分化相关基因5（MDA5）抗体阳性皮肌炎（DM）伴肺间质纤维化（ILD）患者疗效及安全性。方法 回顾性分析2014年6月—2023年6月普洱市人民医院诊断的MDA5阳性DM伴ILD患者40例,其中治疗组20例,采用血浆置换联合PDN 1 mg/kg每日1次口服,4周后减量,每周减总量10%,CTX 1 g每月1次静脉滴注,共6次治疗;另20例设为对照组,采用PDN 1 mg/kg每日1次口服,4周后减量,每周减总量10%,CTX 1 g每月1次静脉滴注,共6次治疗,分别于治疗后3月、6月检测一氧化碳弥散量（DLCO）、第1秒用力呼气量（FEV₁）,血清铁蛋白（SF）、C-反应蛋白（CRP）、涎液化糖链抗原（KL-6）、MDA5转阴率行疗效评估。结果 在治疗3个月和6个月时,两组患者的DLCO、FEV1、SF、CRP、KL-6、MDA5转阴率等指标的完全缓解率不一致。其中,3个月时,治疗组上述指标的完全缓解率依次为95%、85%、90%、90%、90%、85%,对照组依次为15%、20%、20%、15%、0%、0%。两组患者在治疗3个月的DLCO、FEV1、SF、CRP、KL-6水平和MDA5转阴数均有所不同。其中治疗组的DLCO、KL-6、CRP水平均较对照组降低（P<0.01）,治疗组FEV₁水平较对照组升高（P<0.01）,治疗组SF水平较对照组降低（P<0.05）,两组治疗6个月时,治疗组上述指标缓解率依次为95%、85%、90%、90%、90%、85%,对照组依次为20%、25%、20%、20%、20%、5%。两组患者在DLCO、FEV₁、SF、CRP、KL-6水平以及MDA5转阴数和死亡例数方面比较差异均有统计学意义,其中治疗组的DLCO、KL-6和CRP水平均较对照组降低（P<0.01）,治疗组FEV₁水平较对照组升高（P<0.01）,SF水平治疗组较对照组降低（P<0.05）。结论 在MDA5抗体阳性DM伴ILD患者治疗中,给予血浆置换联合PDN、CTX治疗,可以提高疗效,降低病死率。

Objective To explore the effect of plasmapheresis combined with prednisone（PDN）plus cytoxan（CTX）on patients with anti-melanoma differentiation-associated gene 5（MDA 5）antibody-positive dermatomyositis（DM）with interstitial lung disease（ILD）. Methods The data of 40 patients with MDA 5 positive DM and ILD diagnosed in the People's Hospital of Pu'er City from June 2014 to June 2023 were retrospectively was analyzed．Twenty patients of the treatment group were treated with plasmapheresis combined with PDN 1mg / kg once daily,which was reduced by 10% per week after 4 weeks．The other 20 patients of the control group were treated with PDN 1mg / kg once daily,which was reduced after 4 weeks by 10% per week,and CTX 1g once per month．diffusing capacity of the lungs for carbon monoxide（DLCO）,forced expiratory volume in the first second（FEV₁）,serum ferritin（SF）,C-reactive protein（CRP）,Krebs Von den Lungen-6（KL-6）and MDA5 negative conversion rate were measured at 3 and 6 months after treatment,respectively. Results At 3 and 6 months of treatment,complete remission rates of DLCO,FEV₁,SF,CRP,KL-6,MDA 5 conversion and other indicators were inconsistent．Among them,at 3 months,the complete response rate of the above indicators in the treatment group was successively：95%,85%,90%,90%,90% and 85%．The control group was 15%,20%,20%,15%,and 0%,0%．Statistical analysis showed that the levels of DLCO,FEV₁,SF,CRP,KL-6 and MDA 5 significantly varied at 3 months of treatment．Pairwise comparison of LSD found that the DLCO,KL-6 and CRP levels in the treatment group were significantly lower than the control group（P<0.01）,the FEV₁ level in the treatment group was significantly higher（P<0.01）,and the SF level in the treatment group was significantly lower（P<0.05）．After 6 month of treatment,the complete response rate of the above indicators in the treatment group were 95%,85%,90%,90%,90% and 85%,and the complete response rate of the above indicators in the control group was 20%,25%,20%,20%,20% and 5%．Statistical analysis showed the levels of DLCO,FEV₁,SF,CRP,KL-6 for the amount of MDA 5 and the number of deaths between two groups were significantly different．Further pairwise comparison of LSD showed that the DLCO,KL-6 and CRP levels in the treatment group were significantly lower compared with the control group（P<0.01）,the FEV₁ level was significantly increased compared with the control group（P<0.01）,and the SF treatment group was significantly decreased compared with the control group（P<0.05）. Conclusions In the treatment of patients with MDA 5 antibody positive DM with ILD,the treatment of plasmapheresis combined with PDN and CTX can significantly improve the efficacy and reduce the mortality rate．