BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlyin...BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.展开更多
目的 基于循证医学,构建一套适用于进行性纤维化间质性肺病患者的规范化肺康复训练方案,丰富肺康复指导的系统教育方案内涵,为临床康复护理提供科学指导。方法 计算机检索中国WanFang数据库、中国知网数据库(CNKI)、维普数据库(VIP)、...目的 基于循证医学,构建一套适用于进行性纤维化间质性肺病患者的规范化肺康复训练方案,丰富肺康复指导的系统教育方案内涵,为临床康复护理提供科学指导。方法 计算机检索中国WanFang数据库、中国知网数据库(CNKI)、维普数据库(VIP)、中国生物医学文献数据库(CBM)、PubMed、Embase、Cochrane Library、Web of Science数据库文献及指南网站,以循证医学为指导,通过文献回顾分析,采用《临床指南研究与评价系统》(Appraisal of Guidelines for Research and Evaluation,AGREEⅡ)评价指南质量及澳大利亚JBI(Joanna Briggs Institute)循证卫生保健中心开发的文献质量评价工具对随机对照试验、系统评价、类实验文献进行质量评价,对纳入的文献进行全文阅读及等级划分,以汇总最佳证据,拟定进行性纤维化间质性肺病患者肺康复训练方案初稿,最后结合专家会议和专家咨询结果,对方案初稿进行可用性评鉴,最终修订完善方案。结果 经文献质量评价及筛选后,共纳入30篇文献,其中指南与专家共识3篇,随机对照试验文献16篇,系统评价文献6篇,类实验文献5篇。方案内容包括训练评估、呼吸训练、运动训练、健康教育、社会心理干预、营养支持6个一级指标。结论 该研究构建的方案结果可靠,其规范性、科学性和安全性高,能够为进行性纤维化间质性肺病患者临床康复护理工作提供参考。展开更多
BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis.Recently,ther...BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis.Recently,there have been increasing reports of PPFE,and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper lobe.However,cases of unilateral PPFE are scarce.CASE SUMMARY A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional dyspnea.The patient’s symptoms started 6 mo previously and had been gradually worsening.At the time of presentation,he felt dyspnea when walking at his own pace.Radiologic findings suggested PPFE,but the lesion was localized in the upper lobe of the right lung.After multidisciplinary discussion,a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis,and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis,which met the histopathologic criteria of definite PPFE.After multidisciplinary discussion in an experienced interstitial lung disease center,we confirmed the diagnosis of unilateral PPFE.Furthermore,we confirmed the progression of PPFE on radiologic findings during the followup period.CONCLUSION Clinicians should consider PPFE,even in cases with unilateral,predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.展开更多
目的探讨抗黑色素瘤分化相关蛋白-5(melanoma differentiation associated protein-5,MDA5)抗体、抗Ro-52抗体双阳性皮肌炎的临床特征及预后情况。方法收集2018年8月~2022年7月空军军医大学第二附属医院唐都医院收治的抗MDA5抗体阳性的4...目的探讨抗黑色素瘤分化相关蛋白-5(melanoma differentiation associated protein-5,MDA5)抗体、抗Ro-52抗体双阳性皮肌炎的临床特征及预后情况。方法收集2018年8月~2022年7月空军军医大学第二附属医院唐都医院收治的抗MDA5抗体阳性的47例皮肌炎患者,根据抗Ro-52抗体是否阳性,分为MDA5+Ro-52阳性组(n=23),MDA5+Ro-52阴性组(n=24),回顾性分析其临床资料,比较两组患者的临床特征、实验室指标、快速进展性肺间质病变发生率及病死率的差异。结果两组比较,MDA5+Ro-52阳性组Gotton疹、声音嘶哑发生率高于MDA5+Ro-52阴性组,差异均有统计学意义(P均<0.05);皮肤破溃、甲周红斑、向阳疹、披肩征、发热、关节疼痛、咽痛等发生率比较,差异均无统计学意义(P均>0.05);MDA5+Ro-52阳性组淋巴细胞计数[0.65(0.50,0.81)×10^(9)/L vs 1.18(0.91,1.63)×10^(9)/L,z=-3.821,P=0.001]、血清白蛋白[33.40(29.40,35.67)g/L vs 37.25(32.65,40.27)g/L,z=-3.325,P=0.001]、动脉氧分压[66.60(58.60,86.80)mmHg vs 88.60(75.67,95.72)mmHg,z=-2.373,P=0.018]、血氧饱和度[90.40%(89.00%,95.00%)vs 94.90%(90.50%,97.73%),z=-2.353,P=0.019]低于MDA5+Ro-52阴性组,差异有统计学意义(P<0.05),红细胞沉降率[41.00(30.00,62.50)mm/h vs 28.50(21.50,48.75)mm/h,z=2.161,P=0.031]、血清乳酸脱氢酶水平[426.00(335.50,605.50)U/L vs 260.00(217.50,373.25)U/L,z=3.313,P=0.011]、血清铁蛋白水平[1210.00(465.50,2749.00)μg/L vs 366.00(150.25,629.25)μg/L,z=2.856,P=0.004]、快速进展性肺间质病变发生率(73.91%vs 25.00%,χ^(2)=11.245,P=0.001)及病死率(43.47%vs 8.33%,χ^(2)=7.63,P=0.006)均高于抗MDA5+Ro-52阴性组,差异有统计学意义(P<0.05)。结论抗MDA5抗体、抗Ro-52抗体双阳性的皮肌炎患者易出现血清乳酸脱氢酶、铁蛋白水平增高,血清白蛋白、外周血淋巴细胞计数减低,且更易合并快速进展性肺间质病变、出现低氧血症,预后差,病死率高,需引起临床医师的重视。展开更多
目的探讨血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗黑色素瘤分化相关基因-5(melanoma differentiation-associated gene 5,MDA5)抗体阳性皮肌炎合并快速进展性间质性肺病的疗效和安全性。方法回顾性分析2016年3月~2018年11...目的探讨血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗黑色素瘤分化相关基因-5(melanoma differentiation-associated gene 5,MDA5)抗体阳性皮肌炎合并快速进展性间质性肺病的疗效和安全性。方法回顾性分析2016年3月~2018年11月在河北医科大学附属石家庄平安医院治疗的抗MDA5阳性皮肌炎合并快速进展性间质性肺病12例患者,均使用HA280树脂灌流器进行血液灌流治疗,同时联合应用甲泼尼龙、他克莫司和/或环磷酰胺等药物。收集患者治疗前后外周血淋巴细胞计数、白介素(interleukin,IL)-6、IL-10、C反应蛋白、肺泡Ⅱ型细胞表面抗原-6(Krebs Von den Lungen-6,KL-6)、铁蛋白、动脉血氧分压、动脉血二氧化碳分压、肺部高分辨CT评分等指标。治疗前、治疗后2周及4周间比较采用方差分析,两两比较采用LSD-t检验。结果2例死亡,其余10例患者随访至今存活。经过4周治疗与治疗前对比,血清IL-6、IL-10、KL-6、铁蛋白、C反应蛋白明显下降(F值分别为6.593,9.029,7.872,8.760,20.490;P值分别为0.005,0.001,0.002,0.001,<0.001),外周血淋巴细胞计数、动脉血氧分压、动脉血二氧化碳分压明显改善(F值分别为16.810,3.580,3.467;P值分别为<0.001,0.042,0.046),肺部高分辨CT评分改善(F=2.073,P=0.145),但无统计学意义。结论血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗MDA5抗体阳性皮肌炎合并快速进展性间质性肺病具有较好疗效,且安全性较好。展开更多
文摘BACKGROUND Anti-melanoma differentiation-associated gene 5 antibody-positive(anti-MDA5 Ab+)dermatomyositis complicated with rapidly progressive interstitial lung disease(anti-MDA5 Ab+DM-RP-ILD)has an unclear underlying mechanism with no recommended unified treatment plan.Herein,one of the cases that we report(Case 2)was successfully treated with tocilizumab despite having lung infection.CASE SUMMARY Case 1 was a 30-year-old woman who was admitted due to recurrent rash for 5 mo,fever and cough for 1 mo,and chest tightness for 3 d.She was diagnosed with non-myopathic dermatomyositis(anti-MDA5 Ab+)and interstitial pneumonia,and was treated with the combination of hormone therapy and cyclophosphamide followed by oral tacrolimus.Case 2 was a 31-year-old man admitted due to systemic rash accompanied by muscle weakness of limbs for more than 1 mo,and chest tightness and dry cough for 4 d.He was diagnosed with dermatomyositis(anti-MDA5 Ab+)and acute interstitial pneumonia with Pneumocystis jirovecii and Aspergillus fumigatus infections and was treated with hormone therapy(without cyclophosphamide)and the combination of tocilizumab and tacrolimus.The condition of both patients eventually improved and they were discharged and showed clinically stable condition at the latest follow-up.CONCLUSION Tocilizumab could be a salvage treatment for patients with anti-MDA5 Ab+DMRP-ILD who are refractory to intensive immunosuppression.
文摘目的 基于循证医学,构建一套适用于进行性纤维化间质性肺病患者的规范化肺康复训练方案,丰富肺康复指导的系统教育方案内涵,为临床康复护理提供科学指导。方法 计算机检索中国WanFang数据库、中国知网数据库(CNKI)、维普数据库(VIP)、中国生物医学文献数据库(CBM)、PubMed、Embase、Cochrane Library、Web of Science数据库文献及指南网站,以循证医学为指导,通过文献回顾分析,采用《临床指南研究与评价系统》(Appraisal of Guidelines for Research and Evaluation,AGREEⅡ)评价指南质量及澳大利亚JBI(Joanna Briggs Institute)循证卫生保健中心开发的文献质量评价工具对随机对照试验、系统评价、类实验文献进行质量评价,对纳入的文献进行全文阅读及等级划分,以汇总最佳证据,拟定进行性纤维化间质性肺病患者肺康复训练方案初稿,最后结合专家会议和专家咨询结果,对方案初稿进行可用性评鉴,最终修订完善方案。结果 经文献质量评价及筛选后,共纳入30篇文献,其中指南与专家共识3篇,随机对照试验文献16篇,系统评价文献6篇,类实验文献5篇。方案内容包括训练评估、呼吸训练、运动训练、健康教育、社会心理干预、营养支持6个一级指标。结论 该研究构建的方案结果可靠,其规范性、科学性和安全性高,能够为进行性纤维化间质性肺病患者临床康复护理工作提供参考。
文摘BACKGROUND Pleuroparenchymal fibroelastosis(PPFE)is a rare idiopathic interstitial pneumonia characterized by predominantly upper lobe involvement with pleural fibrosis and subjacent parenchymal fibrosis.Recently,there have been increasing reports of PPFE,and PPFE might coexist with other interstitial lung diseases in the lower lobe and upper lobe.However,cases of unilateral PPFE are scarce.CASE SUMMARY A 75-year-old Korean male presented to our hospital with chronic dry cough and exertional dyspnea.The patient’s symptoms started 6 mo previously and had been gradually worsening.At the time of presentation,he felt dyspnea when walking at his own pace.Radiologic findings suggested PPFE,but the lesion was localized in the upper lobe of the right lung.After multidisciplinary discussion,a transbronchial lung biopsy in the right upper lobe revealed collapsed alveoli with parenchymal fibroelastosis,and elastic van Gieson staining demonstrated septal elastosis with intra-alveolar collagenosis,which met the histopathologic criteria of definite PPFE.After multidisciplinary discussion in an experienced interstitial lung disease center,we confirmed the diagnosis of unilateral PPFE.Furthermore,we confirmed the progression of PPFE on radiologic findings during the followup period.CONCLUSION Clinicians should consider PPFE,even in cases with unilateral,predominantly upper lung involvement in interstitial lung disease patients through multidisciplinary discussion.
文摘目的探讨抗黑色素瘤分化相关蛋白-5(melanoma differentiation associated protein-5,MDA5)抗体、抗Ro-52抗体双阳性皮肌炎的临床特征及预后情况。方法收集2018年8月~2022年7月空军军医大学第二附属医院唐都医院收治的抗MDA5抗体阳性的47例皮肌炎患者,根据抗Ro-52抗体是否阳性,分为MDA5+Ro-52阳性组(n=23),MDA5+Ro-52阴性组(n=24),回顾性分析其临床资料,比较两组患者的临床特征、实验室指标、快速进展性肺间质病变发生率及病死率的差异。结果两组比较,MDA5+Ro-52阳性组Gotton疹、声音嘶哑发生率高于MDA5+Ro-52阴性组,差异均有统计学意义(P均<0.05);皮肤破溃、甲周红斑、向阳疹、披肩征、发热、关节疼痛、咽痛等发生率比较,差异均无统计学意义(P均>0.05);MDA5+Ro-52阳性组淋巴细胞计数[0.65(0.50,0.81)×10^(9)/L vs 1.18(0.91,1.63)×10^(9)/L,z=-3.821,P=0.001]、血清白蛋白[33.40(29.40,35.67)g/L vs 37.25(32.65,40.27)g/L,z=-3.325,P=0.001]、动脉氧分压[66.60(58.60,86.80)mmHg vs 88.60(75.67,95.72)mmHg,z=-2.373,P=0.018]、血氧饱和度[90.40%(89.00%,95.00%)vs 94.90%(90.50%,97.73%),z=-2.353,P=0.019]低于MDA5+Ro-52阴性组,差异有统计学意义(P<0.05),红细胞沉降率[41.00(30.00,62.50)mm/h vs 28.50(21.50,48.75)mm/h,z=2.161,P=0.031]、血清乳酸脱氢酶水平[426.00(335.50,605.50)U/L vs 260.00(217.50,373.25)U/L,z=3.313,P=0.011]、血清铁蛋白水平[1210.00(465.50,2749.00)μg/L vs 366.00(150.25,629.25)μg/L,z=2.856,P=0.004]、快速进展性肺间质病变发生率(73.91%vs 25.00%,χ^(2)=11.245,P=0.001)及病死率(43.47%vs 8.33%,χ^(2)=7.63,P=0.006)均高于抗MDA5+Ro-52阴性组,差异有统计学意义(P<0.05)。结论抗MDA5抗体、抗Ro-52抗体双阳性的皮肌炎患者易出现血清乳酸脱氢酶、铁蛋白水平增高,血清白蛋白、外周血淋巴细胞计数减低,且更易合并快速进展性肺间质病变、出现低氧血症,预后差,病死率高,需引起临床医师的重视。
文摘目的探讨血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗黑色素瘤分化相关基因-5(melanoma differentiation-associated gene 5,MDA5)抗体阳性皮肌炎合并快速进展性间质性肺病的疗效和安全性。方法回顾性分析2016年3月~2018年11月在河北医科大学附属石家庄平安医院治疗的抗MDA5阳性皮肌炎合并快速进展性间质性肺病12例患者,均使用HA280树脂灌流器进行血液灌流治疗,同时联合应用甲泼尼龙、他克莫司和/或环磷酰胺等药物。收集患者治疗前后外周血淋巴细胞计数、白介素(interleukin,IL)-6、IL-10、C反应蛋白、肺泡Ⅱ型细胞表面抗原-6(Krebs Von den Lungen-6,KL-6)、铁蛋白、动脉血氧分压、动脉血二氧化碳分压、肺部高分辨CT评分等指标。治疗前、治疗后2周及4周间比较采用方差分析,两两比较采用LSD-t检验。结果2例死亡,其余10例患者随访至今存活。经过4周治疗与治疗前对比,血清IL-6、IL-10、KL-6、铁蛋白、C反应蛋白明显下降(F值分别为6.593,9.029,7.872,8.760,20.490;P值分别为0.005,0.001,0.002,0.001,<0.001),外周血淋巴细胞计数、动脉血氧分压、动脉血二氧化碳分压明显改善(F值分别为16.810,3.580,3.467;P值分别为<0.001,0.042,0.046),肺部高分辨CT评分改善(F=2.073,P=0.145),但无统计学意义。结论血液灌流联合甲泼尼龙、他克莫司和/或环磷酰胺等治疗抗MDA5抗体阳性皮肌炎合并快速进展性间质性肺病具有较好疗效,且安全性较好。
