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Increased neutrophil elastase in affected lobes of bronchiectasis and correlation of its levels between sputum and bronchial lavage fluid. 支气管扩张受累肺叶中性粒细胞弹性酶增高及其痰液和支气管灌洗液水平的相关性
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-14 DOI: 10.4046/trd.2024.0078
Lam Nguyen-Ho, Hoang Kim Tu Trinh, Vu Le-Thuong, Kieu Minh Le, Van Thanh Niem Vo, Diem My Vu, Ngoc Tran-Van, James D Chalmers

Background: Neutrophil elastase (NE) has been proposed as a potential biomarker for evaluating the severity and prognosis of bronchiectasis. This study aimed to compare bronchial lavage quantification of NE levels and activities with those of sputum.

Methods: A cross-sectional study was conducted in which 24 Vietnamese adults with bronchiectasis were enrolled from June 2023 to August 2023. All participants underwent bronchoscopy to collect bronchial lavage fluid (BLF) from two bronchial locations: one in the region with the greatest bronchial dilatation and one in the normal bronchi or in patients with all lobes affected, the least abnormal lobe (ABLF and NBLF, respectively). Spontaneously expectorated sputum was also collected.

Results: Out of 24 cases, the prevalence of mild, moderate and severe bronchiectasis was 14/24 (58.4%), 5/24 (20.8%), and 5/24 (20.8%), respectively. NE concentration and activity were significantly higher in sputum and ABLF than in NBLF (p < 0.001). Sputum and ABLF were highly correlated (r = 0.841, p < 0.001) with no significant difference in NE activity between sputum and ABLF. Higher levels of NE activity were seen in more severe bronchiectasis than in mild bronchiectasis in all samples but were only statistically significant for NE activity in sputum (r = 0.418, p = 0.042).

Conclusion: NE activity and concentration are elevated in areas of the lung most affected by bronchiectasis. Sputum is a valid surrogate of pulmonary NE levels, as they correlate strongly with ABLF and confirm in a Vietnamese population the relationship between NE activity and disease severity.

研究背景:中性粒细胞弹性蛋白酶(Neutrophil elastase, NE)已被认为是评估支气管扩张严重程度和预后的潜在生物标志物。本研究旨在比较支气管灌洗与痰液中NE水平和活性的定量。方法:在2023年6月至2023年8月期间,对24名越南成人支气管扩张患者进行了横断面研究。所有参与者都接受了支气管镜检查,从两个支气管位置收集支气管灌洗液(BLF):一个在支气管扩张最大的区域,一个在正常支气管或所有肺叶受影响的患者中,异常肺叶最小(分别为ABLF和NBLF)。同时采集自发痰液。结果:24例患者中,轻、中、重度支气管扩张的患病率分别为14/24(58.4%)、5/24(20.8%)、5/24(20.8%)。痰液和ABLF中NE浓度和活性显著高于NBLF (p < 0.001)。痰液与ABLF高度相关(r = 0.841, p < 0.001),痰液与ABLF间NE活性无显著差异。在所有样本中,重度支气管扩张患者的NE活性水平高于轻度支气管扩张患者,但痰中NE活性仅具有统计学意义(r = 0.418, p = 0.042)。结论:NE活性和浓度在支气管扩张最严重的肺区升高。痰液是肺部NE水平的有效替代品,因为它们与ABLF密切相关,并且在越南人群中证实了NE活动与疾病严重程度之间的关系。
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引用次数: 0
Bibliometric Analysis of Nontuberculous Mycobacteria Research in South Korea. 韩国非结核分枝杆菌研究的文献计量分析。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-14 DOI: 10.4046/trd.2024.0158
Geunin Lee, Young Ae Kang, Youngmok Park

Background: Current research on nontuberculous mycobacteria (NTM) is multidisciplinary; thus, proper organization is necessary to obtain comprehensive insights. Therefore, a bibliometric analysis was performed to identify NTM research characteristics in South Korea.

Methods: The Web of Science was searched for NTM articles authored by Koreans at Korean institutions until March 2023. We collected data on authors, publication year, article type, study design, research area, citations, research institutes, and funding sources for analysis.

Results: Of the 28,092 articles on NTM, Koreans authored 868. After excluding 167 unrelated studies, 701 relevant articles were analyzed. The first study was from 1992, with publication rates markedly increasing from 2004 onwards. Basic research constituted 41.3% (n=290) of papers, whereas clinical research represented 44.7% (n=313). Basic research consisted mostly of biochemistry studies (n=74, 10.6%), while clinical research primarily involved retrospective studies (n=113, 16.1%). Median citations were highest for systematic reviews and meta-analyses (median 23, interquartile range 15-93). Fifty-four institutions participated in NTM research, with the top five contributing to 71% (n=498) of the publications. The National Research Foundation of Korea was the most significant funding source, supporting 181 studies (32.5% of funded articles).

Conclusion: The quality and quantity of NTM research in South Korea has improved. However, it was concentrated within a few institutions and largely funded by few sources. Future research should use more diverse funding sources, be conducted in more institutions, and prioritize prospective study designs to enhance the understanding and treatment of NTM.

背景:目前对非结核分枝杆菌(NTM)的研究是多学科的;因此,要获得全面的见解,适当的组织是必要的。因此,本文采用文献计量学分析来确定韩国NTM研究的特征。方法:到2023年3月,在Web of Science检索韩国机构中韩国人撰写的NTM文章。我们收集了作者、出版年份、文章类型、研究设计、研究领域、引文、研究机构和资金来源等数据进行分析。结果:在NTM的28,092篇文章中,韩国人撰写了868篇。在排除167项不相关研究后,分析了701篇相关文章。第一项研究从1992年开始,从2004年开始,发表率显著增加。基础研究占41.3% (n=290),临床研究占44.7% (n=313)。基础研究以生物化学研究为主(n=74, 10.6%),临床研究以回顾性研究为主(n=113, 16.1%)。系统评价和荟萃分析的引用中位数最高(中位数23,四分位数范围15-93)。54家机构参与了NTM研究,其中排名前五的机构发表了71% (n=498)的论文。国立科学研究财团是最大的资金来源,资助了181项研究(占资助文章的32.5%)。结论:韩国NTM研究的质量和数量都有所提高。然而,它集中在几个机构内,资金来源也很少。未来的研究应使用更多样化的资金来源,在更多的机构进行,并优先考虑前瞻性研究设计,以增强对NTM的理解和治疗。
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引用次数: 0
Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Connective Tissue Disease Associated Interstitial Lung Disease. 韩国肺间质性疾病的诊断和治疗指南:结缔组织疾病相关的肺间质性疾病。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-10 DOI: 10.4046/trd.2024.0148
Ju Hyun Oh, Jae Ha Lee, Sung Jun Chung, Young Seok Lee, Tae-Hyeong Kim, Tae-Jung Kim, Joo Hun Park

Connective tissue disease (CTD) comprising a various range of autoimmune disorders is often accompanied by lung involvement, which can lead to life-threatening complications. The main types of CTDs that can present as interstitial lung disease (ILD) include rheumatoid arthritis, systemic sclerosis, Sjögren's syndrome, mixed connective tissue disease, idiopathic inflammatory myopathies, and systemic lupus erythematosus. CTD-ILD poses a significant challenge in clinical diagnosis and management due to its heterogeneous nature and variable prognosis. Early diagnosis by clinical, serological, and radiographic assessments is crucial to differentiate CTD-ILD from idiopathic forms and to adopt appropriate therapeutic strategies. Therefore, we have reviewed the numerous clinical manifestations and diagnostic approaches for each type of CTD-ILD, considering the diversity and complexity of CTD-ILD. The significance of a multidisciplinary approach in optimizing the management of CTD-ILD has been underscored by recent therapeutic advancements, which include the use of immunosuppressive agents, antifibrotic therapies, and newer biological agents targeting specific pathways implicated in the pathogenesis. Therapeutic strategies should be tailored to each condition, considering the type of CTD, the extent of lung involvement, and the presence of extrapulmonary manifestations. Furthermore, we aimed to provide clinical guidance including therapeutic recommendations for the effective management of CTD-ILD, based on PICO analysis.

结缔组织病(CTD)包括各种自身免疫性疾病,常伴有肺部受累,可导致危及生命的并发症。可表现为间质性肺病(ILD)的主要CTDs类型包括类风湿关节炎、系统性硬化症、Sjögren综合征、混合性结缔组织病、特发性炎症性肌病和系统性红斑狼疮。CTD-ILD因其异质性和预后多变,对临床诊断和治疗提出了重大挑战。通过临床,血清学和放射学评估的早期诊断对于区分CTD-ILD与特发性形式并采取适当的治疗策略至关重要。因此,考虑到CTD-ILD的多样性和复杂性,我们回顾了每种类型CTD-ILD的众多临床表现和诊断方法。最近的治疗进展强调了多学科方法在优化CTD-ILD管理中的重要性,包括使用免疫抑制剂、抗纤维化疗法和针对与发病机制相关的特定途径的新生物制剂。考虑到CTD的类型、肺部受累程度和肺外表现的存在,治疗策略应针对每种情况量身定制。此外,我们旨在提供临床指导,包括基于PICO分析的有效管理CTD-ILD的治疗建议。
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引用次数: 0
Understanding of patients with severe COVID-19 using lung ultrasound. 肺部超声对重症COVID-19患者的了解。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-06 DOI: 10.4046/trd.2024.0025
Seo-Hee Yang, Eun Ju Park, Jung-Hyun Kim, Jin Woo Song, Young-Jae Cho

Background: Lung ultrasound (LUS) has proven valuable in the initial assessment of coronavirus disease 2019 (COVID-19), but its role in detecting pulmonary fibrosis following intensive care remains unclear. This study aims to assess the presence of pulmonary sequelae and fibrosis-like changes using LUS in survivors of severe COVID-19 pneumonia one month after discharge.

Methods: We prospectively enrolled patients with severe COVID-19 who required mechanical ventilation in the ICU and conducted LUS assessments from admission to the outpatient visit after discharge. We tracked changes in key LUS findings and applied our proprietary LUS scoring system. To evaluate LUS accuracy, we correlated measured LUS values with CT scores.

Results: We evaluated B-line presence, pleural thickness, and consolidation in 14 eligible patients. The LUS scores exhibited minimal changes, with values of 19.1, 19.2, and 17.5 at admission, discharge, and the outpatient visit, respectively. Notably, the number of B-lines decreased significantly, from 1.92 at admission to 0.56 at the outpatient visit (p < 0.05), while pleural thickness increased significantly, from 2.05 at admission to 2.48 at the outpatient visit (p < 0.05).

Conclusions: This study demonstrates that lung ultrasound (LUS) can track changes in lung abnormalities in severe COVID-19 patients from ICU admission through to outpatient follow-up. While pleural thickening and B-line patterns showed significant changes, no correlation was found between LUS and HRCT fibrosis scores. These findings suggest that LUS may serve as a supplementary tool for assessing pulmonary recovery in severe COVID-19 cases.

背景:肺超声(LUS)已被证明在2019冠状病毒病(COVID-19)的初步评估中有价值,但其在重症监护后肺纤维化检测中的作用尚不清楚。本研究旨在评估重症COVID-19肺炎患者出院后1个月使用LUS是否存在肺后遗症和纤维化样改变。方法:前瞻性纳入重症监护室需要机械通气的重症COVID-19患者,从入院到出院后门诊进行LUS评估。我们跟踪关键LUS发现的变化,并应用我们专有的LUS评分系统。为了评估LUS的准确性,我们将测量的LUS值与CT评分相关联。结果:我们评估了14例符合条件的患者的b线存在、胸膜厚度和实变。LUS评分变化最小,入院、出院和门诊时分别为19.1、19.2和17.5。值得注意的是,b线数量明显减少,从入院时的1.92下降到门诊时的0.56 (p < 0.05),胸膜厚度明显增加,从入院时的2.05增加到门诊时的2.48 (p < 0.05)。结论:本研究表明肺部超声(LUS)可以跟踪重症COVID-19患者从ICU入院到门诊随访期间肺部异常的变化。虽然胸膜增厚和b线模式显示了显著的变化,但LUS和HRCT纤维化评分之间没有相关性。这些研究结果表明,LUS可作为评估COVID-19重症病例肺恢复情况的补充工具。
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引用次数: 0
Clinical Characteristics of Chronic Obstructive Pulmonary Disease according to Smoking Status. 根据吸烟状况确定慢性阻塞性肺病的临床特征。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-10-30 DOI: 10.4046/trd.2024.0060
Joo Hun Park

Chronic obstructive pulmonary disease (COPD) can be caused by various factors, including lung infections, asthma, air pollution, childhood growth disorders, and genetic factors, though smoking is the predominant risk factor. The main pathological mechanisms in COPD involve small airway disease, emphysema, mucus hypersecretion, and vascular disorders. COPD in non-smokers is characterized by a normal 1-second forced expiratory volume decline, equal sex distribution, younger age of onset, fewer comorbidities, milder airflow obstruction, preserved diffusing capacity of the lungs for carbon monoxide, and radiological features such as more air-trapping and less severe emphysema compared to COPD in smokers. Nevertheless, non-smokers with COPD still experience a high prevalence of acute exacerbations, nearly equal to that of smokers with COPD. Moreover, COPD itself is an independent risk factor for developing lung cancer, regardless of smoking status. Given that COPD coexists with numerous comorbidities, effectively managing these comorbidities is crucial, requiring multifaceted efforts for comprehensive treatment.

慢性阻塞性肺病可由多种因素引起,包括肺部感染、哮喘、空气污染、儿童生长障碍和遗传因素,但吸烟是主要的危险因素。慢性阻塞性肺病的主要病理机制包括小气道疾病、肺气肿、粘液分泌过多和血管病变。与吸烟者的慢性阻塞性肺病相比,非吸烟者的慢性阻塞性肺病具有以下特点:FEV1 下降正常、性别分布均匀、发病年龄较小、合并症较少、气流阻塞较轻、肺部对一氧化碳的弥散能力(DLCO)正常,以及更多的空气截留和较轻的肺气肿等放射学特征。非吸烟者慢性阻塞性肺病的急性加重率仍然很高,几乎与吸烟者的慢性阻塞性肺病相同。此外,无论吸烟与否,慢性阻塞性肺病本身就是罹患肺癌的独立风险因素。考虑到慢性阻塞性肺病与多种并发症并存,有效治疗这些并发症至关重要,需要多方面的努力来综合治疗慢性阻塞性肺病。
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引用次数: 0
High-Dose Rifampicin for 3 Months after Culture Conversion for Drug-Susceptible Pulmonary Tuberculosis. 对药物敏感的肺结核进行培养转换后,大剂量利福平治疗 3 个月。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-09-27 DOI: 10.4046/trd.2024.0099
Nakwon Kwak, Joong-Yub Kim, Hyung-Jun Kim, Byoung-Soo Kwon, Jae Ho Lee, Jeongha Mok, Yong-Soo Kwon, Young Ae Kang, Youngmok Park, Ji Yeon Lee, Doosoo Jeon, Jung-Kyu Lee, Jeong Seong Yang, Jake Whang, Kyung Jong Kim, Young Ran Kim, Minkyoung Cheon, Jiwon Park, Seokyung Hahn, Jae-Joon Yim

Background: This study aimed to determine whether a shorter high-dose rifampicin regimen is non-inferior to the standard 6-month tuberculosis regimen.

Methods: This multicenter, randomized, open-label, non-inferiority trial enrolled participants with respiratory specimen positivity by Xpert MTB/RIF assay or Mycobacterium tuberculosis culture without rifampicin-resistance. Participants were randomized at 1:1 to the investigational or control group. The investigational group received high-dose rifampicin (30 mg/kg/day), isoniazid, and pyrazinamide until culture conversion, followed by high-dose rifampicin and isoniazid for 12 weeks. The control group received the standard 6-month regimen. The primary outcome was the rate of unfavorable outcomes at 18 months post-randomization. The non-inferiority margin was set at <6% difference in unfavorable outcomes rates. The study is registered with ClinicalTrials.gov (NCT04485156).

Results: Between 4 November 2020 and 3 January 2022, 76 participants were enrolled. Of these, 58 were included in the modified intention-to-treat analysis. Unfavorable outcomes occurred in 10 (31.3%) of 32 in the control group and 10 (38.5%) of 26 in the investigational group. The difference was 7.2% (95% confidence interval, ∞ to 31.9%), failing to prove non-inferiority. Serious adverse events and grade 3 or higher adverse events did not differ between the groups.

Conclusion: The shorter high-dose rifampicin regimen failed to demonstrate non-inferiority but had an acceptable safety profile.

背景:本研究旨在确定较短的大剂量利福平治疗方案是否优于标准的 6 个月结核病治疗方案:本研究旨在确定较短的大剂量利福平治疗方案是否优于为期 6 个月的结核病标准治疗方案:这项多中心、随机、开放标签、非劣效试验招募了经 Xpert MTB/RIF 检测或结核分枝杆菌培养呈呼吸道标本阳性且无利福平耐药性的参与者。参与者按 1:1 随机分配到研究组或对照组。研究组接受大剂量利福平(30 毫克/千克/天)、异烟肼和吡嗪酰胺治疗,直到培养结果转阴,然后再接受大剂量利福平和异烟肼治疗 12 周。对照组接受为期 6 个月的标准疗法。主要结果是随机后18个月的不利结果发生率。非劣效性边际设定为 "结果":在 2020 年 11 月 4 日至 2022 年 1 月 3 日期间,共有 76 名参与者入组。其中 58 人被纳入修改后的意向治疗分析。对照组 32 人中有 10 人(31.3%)出现不利结果,研究组 26 人中有 10 人(38.5%)出现不利结果。差异为 7.2%(95% 置信区间,∞ 至 31.9%),未能证明非劣效性。两组的严重不良事件和3级或以上不良事件没有差异:结论:较短的大剂量利福平方案未能证明非劣效性,但其安全性是可以接受的。试验注册 ClinicalTrials.gov NCT04485156。
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引用次数: 0
Korean Guidelines for the Management and Antibiotic Therapy in Adult Patients with Hospital-Acquired Pneumonia. 韩国医院获得性肺炎成人患者管理和抗生素治疗指南》。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-10-11 DOI: 10.4046/trd.2024.0135
Hayoung Choi, Kyung Hoon Min, Young Seok Lee, Youjin Chang, Bo Young Lee, Jee Youn Oh, Ae-Rin Baek, Jongmin Lee, Kyeongman Jeon

Hospital-acquired pneumonia (HAP) and ventilator-associated pneumonia (VAP) are correlated with high morbidity and mortality rates. Guidelines that consider local epidemiologic data are fundamental for identifying optimal treatment strategies. However, Korea has no HAP/VAP guidelines. This study was conducted by a committee of nine experts from the Korean Academy of Tuberculosis and Respiratory Diseases Respiratory Infection Study Group using the results of Korean HAP/VAP epidemiologic studies. Eleven key questions for HAP/VAP diagnosis and treatment were addressed. The Convergence of Opinion on Suggestions and Evidence (CORE) process was used to derive suggestions, and evidence levels and recommendation grades were in accordance with the Grading of Recommendations Assessment Development and Evaluation (GRADE) methodology. Suggestions were made for the 11 key questions pertinent to diagnosis, biomarkers, antibiotics, and treatment strategies for adult patients with HAP/VAP. Using the CORE process and GRADE methodology, the committee generated a series of recommendations for HAP/VAP diagnosis and treatment in the Korean context.

背景:医院获得性肺炎(HAP)和呼吸机相关肺炎(VAP)与高发病率和高死亡率相关。考虑当地流行病学数据的指南是确定最佳治疗策略的基础。然而,韩国尚未制定 HAP/VAP 指南:本研究由韩国结核病和呼吸系统疾病研究院呼吸道感染研究小组的九位专家组成的委员会利用韩国 HAP/VAP 流行病学研究的结果进行。会议讨论了有关 HAP/VAP 诊断和治疗的 11 个关键问题。采用建议与证据意见趋同(CORE)流程得出建议,证据等级和建议等级按照建议评估开发与评价分级(GRADE)方法确定:结果:针对成人 HAP/VAP 患者的诊断、生物标志物、抗生素和治疗策略等 11 个关键问题提出了建议:委员会采用 CORE 流程和 GRADE 方法,为韩国的 HAP/VAP 诊断和治疗提出了一系列建议。
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引用次数: 0
Miliary Tuberculosis Associated with Klebsiella pneumonia: Managing the Double Whammy of Antimicrobial Resistance. 伴有克雷伯氏菌肺炎的睫状体结核病:抗菌药耐药性的双重打击。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-10-30 DOI: 10.4046/trd.2024.0105
Priyavardhan Mishra, Mohit Kondisetti, Anant Patil, Nikhil Sarangdhar, Vijaykumar Gupta
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引用次数: 0
Beyond the Spirometry: New Diagnostic Modalities in Chronic Obstructive Pulmonary Disease. 肺活量测定之外:慢性阻塞性肺病的新诊断模式。
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-09-23 DOI: 10.4046/trd.2024.0040
Jin Hwa Song, Youlim Kim

Spirometry can play a critical role as a gold standard in the diagnosis and treatment of patients with chronic obstructive pulmonary disease (COPD). While the criteria for diagnosis have advanced over time, the Global Initiative for Chronic Obstructive Lung Disease (GOLD) standard of the forced expiratory volume in 1 second/forced vital capacity ratio <0.7 remains the most universally employed metric. However, spirometry cannot be utilized in all situations, and test execution can be difficult for some patients, often showing normal values in the early diagnosis of COPD. Therefore, research on new diagnostic methods is underway. Techniques include whole-body plethysmography for measurement of residual volume and inspiratory capacity and airway resistance, diffusing capacity of carbon monoxide or nitric oxide, impulse oscillometry, infrared time-offlight depth image sensor, diaphragm ultrasonography, which can enable early diagnosis and multifaceted assessment of patients with COPD.

肺活量测定在慢性阻塞性肺疾病(COPD)患者的诊断和治疗中可以作为金标准发挥关键作用。虽然诊断标准随着时间的推移而进步,但全球慢性阻塞性肺疾病倡议(GOLD)的1秒用力呼气量/用力肺活量比<;0.7的标准仍然是最普遍使用的指标。然而,肺活量测定法不能在所有情况下使用,并且对一些患者来说,测试执行可能很困难,通常在COPD的早期诊断中显示正常值。因此,新的诊断方法的研究正在进行中。技术包括测量残气量、吸气量和气道阻力的全身体积脉搏图、一氧化碳或一氧化氮的扩散能力、脉冲振荡法、红外时间飞行深度图像传感器、隔膜超声等,可对COPD患者进行早期诊断和多方面评估。
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引用次数: 0
Idiopathic Inflammatory Myopathies-Associated Interstitial Lung Disease in Adults. 成人特发性炎症性肌病相关间质性肺病
IF 2.5 Q2 RESPIRATORY SYSTEM Pub Date : 2025-01-01 Epub Date: 2024-09-02 DOI: 10.4046/trd.2024.0072
Mitsuhiro Moda, Toyoshi Yanagihara, Ran Nakashima, Hiromitsu Sumikawa, Shigeki Shimizu, Toru Arai, Yoshikazu Inoue

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune diseases characterized by muscle involvement and various extramuscular manifestations. Interstitial lung disease (ILD) is one of the most common extramuscular manifestations of IIM and is associated with significant mortality and morbidity. The clinical phenotypes, treatment responses, and prognosis of IIM-ILD are significantly related to myositis-specific antibody (MSA) profiles, with some racial differences. The features associated with MSA in IIM-ILD could also be relevant to cases of ILD where MSA is present but does not meet the criteria for IIM. The anti-melanoma differentiation-associated gene 5 antibody is highly associated with rapidly progressive ILD (RP-ILD), especially in Asian populations, and with characteristic cutaneous manifestations, such as skin ulcers. Radiologically, ground-glass opacities, consolidations, and nonsegmental linear opacities were more predominant than reticular opacities and honeycombing. While the mortality rate is still around 30%, the prognosis can be improved with early intensive therapy with corticosteroids and multiple immunosuppressants. In contrast, anti-aminoacyl-tRNA synthetase (ARS) antibodies are associated with chronic ILD, although RP-ILD is also common. Patients with anti-ARS antibodies often show lung-predominant presentations, with subtle muscle and skin involvement. Radiologically, reticular opacities, with or without consolidation, are predominant and may progress to honeycombing over time. Combination therapy with corticosteroids and a single immunosuppressant is recommended to prevent relapses, which often lead to a decline in lung function and fatal long-term outcomes. Significant advances in immunology and genetics holds promise for fostering more personalized approaches to managing IIMILD.

特发性炎症性肌病(IIM)是一组以肌肉受累和各种肌外表现为特征的异质性自身免疫性疾病。间质性肺病(ILD)是特发性炎症性肌病最常见的肌肉外表现之一,与严重的死亡率和发病率有关。IIM-ILD的临床表型、治疗反应和预后与肌炎特异性抗体(MSA)谱有很大关系,并存在一些种族差异。IIM-ILD中与MSA相关的特征也可能与存在MSA但不符合IIM标准的ILD病例有关。抗黑色素瘤分化相关基因 5 抗体与快速进展性 ILD(RP-ILD)高度相关,尤其是在亚洲人群中,并与皮肤溃疡等特征性皮肤表现相关。从放射学角度看,磨玻璃不透明、合并症和非节段性线状不透明比网状不透明和蜂窝状不透明更主要。虽然死亡率仍在 30% 左右,但通过早期使用皮质类固醇激素和多种免疫抑制剂进行强化治疗,可以改善预后。相反,抗氨基酸酰-tRNA 合成酶(ARS)抗体与慢性 ILD 有关,尽管 RP-ILD 也很常见。抗ARS抗体患者通常以肺部表现为主,肌肉和皮肤受累较轻。放射学上,网状不透明(伴有或不伴有合并症)是主要表现,随着时间的推移可能发展为蜂窝状。建议使用皮质类固醇和单一免疫抑制剂进行联合治疗,以防止复发,因为复发往往会导致肺功能下降和致命的长期后果。免疫学和遗传学的重大进展有望促进采用更加个性化的方法来管理 IIM-ILD。
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Tuberculosis and Respiratory Diseases
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