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Upfront combination therapy with sildenafil and ambrisentan in patients with chronic thromboembolic pulmonary hypertension. 西地那非和氨布里森坦联合治疗慢性血栓栓塞性肺动脉高压患者。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-12-05 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20250056
William Salibe-Filho, Tulio Martins Vieira, José Leonidas Alves-Junior, Yally Priscila Pessôa Nascimento, Luiza Sarmento Tatagiba, Caio Julio Cesar Fernandes, Carlos Viana Poyares Jardim, Mario Terra-Filho, Rogerio Souza

Objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, being characterized by persistent obstruction of pulmonary vessels and leading to increased pulmonary vascular resistance and right ventricular failure. Although pulmonary endarterectomy is the preferred treatment, medical therapies may offer clinical benefits in specific settings. We sought to evaluate the clinical and hemodynamic response of CTEPH patients treated with sildenafil and ambrisentan upfront combination therapy.

Methods: This was a retrospective cohort study including patients with operable and inoperable CTEPH. The patients were followed from 2019 to 2022 and were treated with sildenafil and ambrisentan as first-line therapy.

Results: Functional and hemodynamic data were analyzed at baseline and after a minimum of six months of therapy. Following treatment, there was a notable improvement in functional class, natriuretic peptide levels, and invasive hemodynamics.

Conclusions: The combined use of sildenafil and ambrisentan appears to be associated with clinical, functional, and hemodynamic improvement in patients with CTEPH.

目的:慢性血栓栓塞性肺动脉高压(CTEPH)是一种罕见的急性肺栓塞并发症,其特征是肺血管持续阻塞,导致肺血管阻力增加和右心室衰竭。虽然肺动脉内膜切除术是首选的治疗方法,但在特定情况下,医学治疗可能会提供临床益处。我们试图评估西地那非和安布里森坦联合治疗CTEPH患者的临床和血流动力学反应。方法:这是一项回顾性队列研究,包括可手术和不可手术的CTEPH患者。患者于2019年至2022年随访,以西地那非和安布里森坦为一线治疗。结果:在基线和至少六个月的治疗后,分析了功能和血流动力学数据。治疗后,功能分级、利钠肽水平和侵入性血流动力学均有显著改善。结论:西地那非和氨布里森坦联合使用似乎与CTEPH患者的临床、功能和血流动力学改善有关。
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引用次数: 0
Risk communication, respiratory health risks, and air pollution forecasting in the city of Rio de Janeiro, Brazil. 巴西里约热内卢市的风险沟通、呼吸健康风险和空气污染预测。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-12-05 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20250168
Kevin Do Hyeon Park, Kevin Cromar, Gina Gonzales, Laura Gladson, Felipe Cerbella Mandarino, Lucia Helena Barros Dos Santos, Bruno Bôscaro França, Noussair Lazrak, Katherine Emma Knowland

Objective: Although communicating air pollution risks is critical for protecting public health, particularly in low- and middle-income countries (LMICs), its effectiveness remains underexplored. This study evaluated current risk communication practices in the city of Rio de Janeiro, Brazil, by assessing the associations between short-term exposure to pollutants and respiratory-related hospital admissions; the ability of the Brazilian national índice de qualidade do ar (IQAr, air quality index) to reflect health risks; and the accuracy of pollutant forecasts in comparison with monitored concentrations.

Methods: Exposure and health data for the 2014-2019 period were obtained through a research partnership with local government officials. Poisson generalized linear models were employed to determine whether IQAr values and short-term exposure to air pollutants, including nitrogen dioxide (NO2) and particulate matter (PM), were associated with daily hospital admissions for respiratory disease. Bias-corrected, forecasted daily concentrations of individual air pollutants from the Goddard Earth Observing System Composition Forecast Composition Forecast (GEOS-CF) model were employed to assess the performance of existing forecasting tools for use in risk communication.

Results: Significant associations were consistently observed between hospital admissions for respiratory disease and short-term exposures to NO2 and coarse PM, with excess risks of 5.1% (95% CI: 1.3-8.9%) and 5.6% (95% CI: 1.5-9.9%), respectively, per interquartile range increases in lag day 0-1 exposures. Values of IQAr were not significantly associated with respiratory health events, likely due to their failure to capture the health risks associated with NO2. Bias-corrected forecasts from the GEOS-CF model showed strong correlations with observed pollutant concentrations.

Conclusions: These findings indicate that adopting a health-based, multi-pollutant index, combined with improved forecasting tools, could substantially strengthen risk communication in the city of Rio de Janeiro and other LMIC settings.

目标:虽然通报空气污染风险对保护公众健康至关重要,特别是在低收入和中等收入国家,但其有效性仍未得到充分探讨。本研究通过评估短期接触污染物与呼吸道相关住院之间的关系,评估了巴西里约热内卢市目前的风险沟通做法;巴西国家空气质量指数índice de qualade do ar (IQAr)反映健康风险的能力;以及污染物预报与监测浓度的准确性。方法:通过与当地政府官员的研究合作,获得2014-2019年期间的暴露和健康数据。使用泊松广义线性模型来确定IQAr值和短期暴露于空气污染物(包括二氧化氮(NO2)和颗粒物(PM))是否与呼吸系统疾病的每日住院率相关。利用戈达德地球观测系统成分预测成分预测(GEOS-CF)模型对个别空气污染物的日浓度进行了偏差校正,以评估用于风险沟通的现有预测工具的性能。结果:呼吸系统疾病住院与短期暴露于二氧化氮和粗颗粒物之间一致观察到显著关联,在滞后0-1天暴露时,每四分位数范围内的超额风险分别为5.1% (95% CI: 1.3-8.9%)和5.6% (95% CI: 1.5-9.9%)。IQAr值与呼吸系统健康事件没有显著相关性,这可能是由于它们未能捕捉到与二氧化氮相关的健康风险。来自GEOS-CF模型的偏差校正预报显示与观测到的污染物浓度有很强的相关性。结论:这些发现表明,采用基于健康的多污染物指数,结合改进的预测工具,可以大大加强巴西里约热内卢市和其他低收入和中等收入国家环境的风险沟通。
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引用次数: 0
Effect of combined strength and endurance training in adults with asthma: a randomized controlled trial. 联合力量和耐力训练对成人哮喘患者的影响:一项随机对照试验。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-12-05 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20250009
Giuseppe Lo Bello, Federico Mattia Oliva, Alberto Malovini, Nicolino Ambrosino, Matteo Tarasconi, Andrea Zanini, Elisabetta Zampogna

Objective: Pulmonary rehabilitation programs, including exercise training, have an established role in the treatment of chronic respiratory diseases but are not routinely used in asthma. Most studies of individuals with asthma have focused on endurance training, and there is therefore limited data available on strength training. The aim of this study was to evaluate the effects that adding strength training to a program of endurance training and education has on the quality of life of such individuals.

Methods: In this single-center, parallel-group randomized controlled trial, adults with moderate-to-severe asthma admitted for in-hospital pulmonary rehabilitation between June of 2021 and October of 2022 were randomized to either a study group (SG) or a control group (CG). The SG received strength training alongside endurance training and education, whereas the CG received the same endurance training and education, along with sham mobility exercise training instead of strength training. The primary outcome was the change in the Asthma Quality of Life Questionnaire (AQLQ) score from hospital admission to discharge.

Results: A total of 61 participants were randomized, with 31 being assigned to the SG and 30 being assigned to the CG. At discharge, the AQLQ score showed significant improvement in both groups (p < 0.001 for the SG and p = 0.02 for the CG), albeit without a significant difference between the groups (p > 0.99). In contrast, peripheral muscle strength improved significantly from admission to discharge only in the SG, with a significant difference between the groups in terms of quadriceps strength (p = 0.03).

Conclusions: Adding strength training to endurance training and education does not seem to result in further improvement in the quality of life of individuals with moderate-to-severe asthma.

目的:肺康复计划,包括运动训练,在慢性呼吸系统疾病的治疗中有既定的作用,但在哮喘中并不常用。大多数对哮喘患者的研究都集中在耐力训练上,因此关于力量训练的数据有限。这项研究的目的是评估在耐力训练和教育项目中加入力量训练对这些人的生活质量的影响。方法:在这项单中心、平行组随机对照试验中,2021年6月至2022年10月期间入院的中度至重度哮喘成人患者被随机分为研究组(SG)或对照组(CG)。SG组在接受耐力训练和教育的同时接受力量训练,而CG组则接受同样的耐力训练和教育,同时接受假活动训练,而不是力量训练。主要观察指标为入院至出院期间哮喘生活质量问卷(AQLQ)评分的变化。结果:共有61名参与者被随机化,其中31名被分配到SG, 30名被分配到CG。出院时,两组AQLQ评分均有显著改善(SG组p < 0.001, CG组p = 0.02),但两组间无显著差异(p < 0.99)。相比之下,从入院到出院,周围肌肉力量仅在SG中有显著改善,在股四头肌力量方面,组间差异显著(p = 0.03)。结论:在耐力训练和教育的基础上增加力量训练似乎并不能进一步改善中重度哮喘患者的生活质量。
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引用次数: 0
Cannabidiol oil-an uncommon cause of exogenous lipoid pneumonia. 大麻二酚油是外源性脂质肺炎的罕见病因。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-12-05 DOI: 10.36416/1806-3756/e20250283
Arnaldo Noronha, Gláucia Zanetti, Edson Marchiori
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引用次数: 0
COPD: comparative study of vaccinated and unvaccinated patients for pneumococcal disease. 慢性阻塞性肺病:肺炎球菌疾病接种疫苗和未接种疫苗患者的比较研究。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-12-05 DOI: 10.36416/1806-3756/e20240369
Adriana de Siqueira Carvalho Knabben, Rosemeri Maurici
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引用次数: 0
Evaluation of screening methods for preclinical interstitial lung disease associated with rheumatoid arthritis. 类风湿关节炎相关临床前间质性肺疾病筛查方法的评价
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-10-27 DOI: 10.36416/1806-3756/e20240402
Ana Luisa Bagno de Almeida, Mariana Drummond Martins Lima, Maria Fernanda B Resende Guimarães, Eliane Viana Mancuzo
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引用次数: 0
Prospective cohort studies and their contribution to public health end evidence-based medicine. 前瞻性队列研究及其对公共卫生的贡献结束循证医学。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-10-27 DOI: 10.36416/1806-3756/e20250355
Gustavo Zabert, Cecilia M Patino, Juliana Carvalho Ferreira
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引用次数: 0
Maximal dynamic inspiratory pressure: S-Index prediction values and diagnosis accuracy. 最大动态吸气压力:s指数预测值及诊断准确性。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-10-27 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20240409
Vitor Costa Souza, Maria Fernanda Lima Souza Saldanha, Eloara Vieira Machado Ferreira, Luiz Eduardo Nery, Priscila Cristina de Abreu Sperandio

Objective: To establish reference values and prediction equations for the strength index (S-Index), in order to meet the growing demand for clinical application and diagnostic understanding of maximal dynamic inspiratory pressure.

Methods: This was a prospective study of 120 healthy subjects between 18 and 80 years of age. The S-Index, measured from RV to TLC after at least eight reproducible maximal maneuvers with a < 10% difference, was obtained. The MIP was also measured, and differences between S-Index and MIP values were analyzed. A multiple linear regression model estimating the S-Index value was based on clinically significant independent variables. For model cross-validation and diagnostic accuracy, we used a separate sample of COVID-19 survivors to compare the observed and predicted S-Index values.

Results: The S-Index strongly correlated with the FEV1 and FVC. However, sex, age, weight, and height retained their significance in all final models, collectively explaining 62% of the variation in the observed values. The performance of the prediction equation was satisfactory in suggesting differences between COVID-19 survivors with an MIP < 80 cmH2O and those with an MIP ≥ 80 cmH2O. For both sexes, the S-Index exhibited the potential for ruling out, rather than confirming, inspiratory muscle weakness. If below the lower limit of normal, further evaluation is important, especially in men.

Conclusions: To our knowledge, this is the first set of reference equations for the S-Index based on a healthy adult population across various age groups in Brazil. Its potential as an adjunct index in evaluating inspiratory muscle strength was also explored for the first time.

目的:建立最大动态吸气压力强度指数(S-Index)的参考值及预测方程,以满足临床应用和诊断认识日益增长的需求。方法:这是一项前瞻性研究,120名年龄在18至80岁之间的健康受试者。s指数,从RV到TLC测量至少8个可重复的最大操作后,差异< 10%,获得。测量MIP,并分析s指数与MIP值的差异。基于具有临床意义的自变量,建立多元线性回归模型估计S-Index值。为了模型交叉验证和诊断准确性,我们使用了单独的COVID-19幸存者样本来比较观察到的和预测的s指数值。结果:s指数与FEV1、FVC呈显著正相关。然而,性别、年龄、体重和身高在所有最终模型中仍然具有重要意义,它们共同解释了观察值中62%的变化。预测方程的性能令人满意,表明MIP < 80 cmH2O的COVID-19幸存者与MIP≥80 cmH2O的幸存者之间存在差异。无论男女,s指数都显示出排除而不是确认吸气肌无力的可能性。如果低于正常的下限,进一步的评估是重要的,特别是在男性。结论:据我们所知,这是第一组基于巴西各年龄段健康成年人的s指数参考方程。本文也首次探讨了其作为评价吸气肌力量的辅助指标的潜力。
{"title":"Maximal dynamic inspiratory pressure: S-Index prediction values and diagnosis accuracy.","authors":"Vitor Costa Souza, Maria Fernanda Lima Souza Saldanha, Eloara Vieira Machado Ferreira, Luiz Eduardo Nery, Priscila Cristina de Abreu Sperandio","doi":"10.36416/1806-3756/e20240409","DOIUrl":"10.36416/1806-3756/e20240409","url":null,"abstract":"<p><strong>Objective: </strong>To establish reference values and prediction equations for the strength index (S-Index), in order to meet the growing demand for clinical application and diagnostic understanding of maximal dynamic inspiratory pressure.</p><p><strong>Methods: </strong>This was a prospective study of 120 healthy subjects between 18 and 80 years of age. The S-Index, measured from RV to TLC after at least eight reproducible maximal maneuvers with a < 10% difference, was obtained. The MIP was also measured, and differences between S-Index and MIP values were analyzed. A multiple linear regression model estimating the S-Index value was based on clinically significant independent variables. For model cross-validation and diagnostic accuracy, we used a separate sample of COVID-19 survivors to compare the observed and predicted S-Index values.</p><p><strong>Results: </strong>The S-Index strongly correlated with the FEV1 and FVC. However, sex, age, weight, and height retained their significance in all final models, collectively explaining 62% of the variation in the observed values. The performance of the prediction equation was satisfactory in suggesting differences between COVID-19 survivors with an MIP < 80 cmH2O and those with an MIP ≥ 80 cmH2O. For both sexes, the S-Index exhibited the potential for ruling out, rather than confirming, inspiratory muscle weakness. If below the lower limit of normal, further evaluation is important, especially in men.</p><p><strong>Conclusions: </strong>To our knowledge, this is the first set of reference equations for the S-Index based on a healthy adult population across various age groups in Brazil. Its potential as an adjunct index in evaluating inspiratory muscle strength was also explored for the first time.</p>","PeriodicalId":14845,"journal":{"name":"Jornal Brasileiro De Pneumologia","volume":"51 4","pages":"e20240409"},"PeriodicalIF":3.0,"publicationDate":"2025-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12602067/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145421817","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Lateral flow urine lipoarabinomannan assay for tuberculosis diagnosis in HIV-positive inpatients and outpatients. 侧流尿脂阿拉伯糖甘露聚糖测定对hiv阳性住院和门诊患者肺结核诊断的价值。
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-10-27 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20250152
Giovana Rodrigues Pereira, Márcia Silva Barbosa, Carina Secchi, Maiara Priscila Dos Passos, André Kulzer Santos, Raimunda Sinthia Lima de Braga, Marina Scheffer de Souza, Gean Souza Ramos, João Vitor Vigne Duz, Denise Rossato Silva

Objective: To evaluate tuberculosis diagnosis with the use of the lateral flow urine lipoarabinomannan assay (LF-LAM) in addition to the Xpert® MTB/RIF Ultra assay on sputum in HIV-positive inpatients and outpatients.

Methods: This was a prospective cross-sectional study including HIV-positive patients ≥ 18 years of age with an indication for LF-LAM in accordance with Brazilian National Ministry of Health criteria.

Results: A total of 140 patients were included in the study. LF-LAM was positive in 23 (16.4%). An additional 12 (8.6%) were diagnosed with the aid of LF-LAM, this increase in tuberculosis detection being statistically significant. LF-LAM-positive patients were younger and had lower CD4 counts in comparison with LF-LAM-negative patients. Smoking was more common among LF-LAM-negative patients than among LF-LAM-positive patients.

Conclusions: The use of LF-LAM significantly increases the detection of tuberculosis in HIV-positive patients, mostly in those who are hospitalized. These findings highlight the utility of LF-LAM, especially in regions with high tuberculosis and HIV infection incidence.

目的:探讨在Xpert®MTB/RIF Ultra检测的基础上,应用侧流尿脂arabinmanannan assay (LF-LAM)对hiv阳性住院和门诊患者痰液的结核诊断价值。方法:这是一项前瞻性横断面研究,纳入年龄≥18岁且符合巴西国家卫生部标准的LF-LAM适应症的hiv阳性患者。结果:共纳入140例患者。LF-LAM阳性23例(16.4%)。另有12例(8.6%)通过LF-LAM诊断,这一增加的结核病检出率具有统计学意义。与lf - lam阴性患者相比,lf - lam阳性患者更年轻,CD4计数更低。吸烟在lf - lam阴性患者中比在lf - lam阳性患者中更常见。结论:LF-LAM的使用显著提高了hiv阳性患者的结核病检出率,主要发生在住院患者中。这些发现强调了LF-LAM的效用,特别是在结核病和艾滋病毒感染率高的地区。
{"title":"Lateral flow urine lipoarabinomannan assay for tuberculosis diagnosis in HIV-positive inpatients and outpatients.","authors":"Giovana Rodrigues Pereira, Márcia Silva Barbosa, Carina Secchi, Maiara Priscila Dos Passos, André Kulzer Santos, Raimunda Sinthia Lima de Braga, Marina Scheffer de Souza, Gean Souza Ramos, João Vitor Vigne Duz, Denise Rossato Silva","doi":"10.36416/1806-3756/e20250152","DOIUrl":"10.36416/1806-3756/e20250152","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate tuberculosis diagnosis with the use of the lateral flow urine lipoarabinomannan assay (LF-LAM) in addition to the Xpert® MTB/RIF Ultra assay on sputum in HIV-positive inpatients and outpatients.</p><p><strong>Methods: </strong>This was a prospective cross-sectional study including HIV-positive patients ≥ 18 years of age with an indication for LF-LAM in accordance with Brazilian National Ministry of Health criteria.</p><p><strong>Results: </strong>A total of 140 patients were included in the study. LF-LAM was positive in 23 (16.4%). An additional 12 (8.6%) were diagnosed with the aid of LF-LAM, this increase in tuberculosis detection being statistically significant. LF-LAM-positive patients were younger and had lower CD4 counts in comparison with LF-LAM-negative patients. Smoking was more common among LF-LAM-negative patients than among LF-LAM-positive patients.</p><p><strong>Conclusions: </strong>The use of LF-LAM significantly increases the detection of tuberculosis in HIV-positive patients, mostly in those who are hospitalized. These findings highlight the utility of LF-LAM, especially in regions with high tuberculosis and HIV infection incidence.</p>","PeriodicalId":14845,"journal":{"name":"Jornal Brasileiro De Pneumologia","volume":"51 4","pages":"e20250152"},"PeriodicalIF":3.0,"publicationDate":"2025-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12602061/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145421750","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical, functional, and computed tomographic characterization of idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective cohort study. 特发性炎性肌病相关间质性肺病的临床、功能和计算机断层扫描特征:一项回顾性队列研究
IF 3 4区 医学 Q2 RESPIRATORY SYSTEM Pub Date : 2025-10-27 eCollection Date: 2025-01-01 DOI: 10.36416/1806-3756/e20250123
José Ricardo Bandeira de Oliveira Filho, André Nathan Costa, Bruno Guedes Baldi, Mark Wanderley, Marcio Valente Yamada Sawamura, Ronaldo Adib Kairalla

Objective: To evaluate clinical, functional, and CT characteristics, as well as disease progression, in patients with idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) treated at a referral center in Brazil.

Methods: This was a retrospective cohort study analyzing demographic characteristics, clinical variables, pulmonary function test results, HRCT findings, serological profiles, treatments, and outcomes.

Results: Seventy-nine IIM-ILD patients were included in the present study. The mean follow-up period was 8.7 ± 4.7 years. The most common diagnosis was antisynthetase syndrome, observed in 51 (64.5%) of the 79 patients. The most common symptoms were dyspnea (in 94.9%), arthralgia (in 82.2%), and muscle weakness (in 75.9%). Mean baseline FVC was 2.19 ± 0.75 L, corresponding to 62.5% of the predicted value. During follow-up, FVC showed significant improvement. The most common CT patterns were indeterminate (in 44.4%) and nonspecific interstitial pneumonia (in 35.4%). Treatment most frequently included prednisone (in 98.7%), azathioprine (in 92.3%), or methotrexate (in 57.7%). Overall survival was 84.8%. Mortality was higher among patients who developed pulmonary hypertension and those who required intravenous methylprednisolone pulse therapy.

Conclusions: Most patients with IIM-ILD progress well with immunosuppressive therapy. Pulmonary hypertension and the need for methylprednisolone pulse therapy appear to be associated with higher mortality.

目的:评估巴西一家转诊中心治疗的特发性炎性肌病相关间质性肺病(IIM-ILD)患者的临床、功能和CT特征以及疾病进展。方法:这是一项回顾性队列研究,分析人口统计学特征、临床变量、肺功能检查结果、HRCT结果、血清学特征、治疗和结局。结果:本研究纳入了79例IIM-ILD患者。平均随访时间8.7±4.7年。最常见的诊断是抗合成酶综合征,79例患者中有51例(64.5%)出现。最常见的症状是呼吸困难(94.9%)、关节痛(82.2%)和肌肉无力(75.9%)。平均基线FVC为2.19±0.75 L,为预测值的62.5%。随访期间,FVC有明显改善。最常见的CT表现为不确定(44.4%)和非特异性间质性肺炎(35.4%)。最常见的治疗包括强的松(98.7%)、硫唑嘌呤(92.3%)或甲氨蝶呤(57.7%)。总生存率为84.8%。发生肺动脉高压和需要静脉注射甲基强的松龙脉冲治疗的患者死亡率更高。结论:大多数IIM-ILD患者经免疫抑制治疗进展良好。肺动脉高压和甲基强的松龙脉冲治疗的需要似乎与较高的死亡率有关。
{"title":"Clinical, functional, and computed tomographic characterization of idiopathic inflammatory myopathy-associated interstitial lung disease: a retrospective cohort study.","authors":"José Ricardo Bandeira de Oliveira Filho, André Nathan Costa, Bruno Guedes Baldi, Mark Wanderley, Marcio Valente Yamada Sawamura, Ronaldo Adib Kairalla","doi":"10.36416/1806-3756/e20250123","DOIUrl":"10.36416/1806-3756/e20250123","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate clinical, functional, and CT characteristics, as well as disease progression, in patients with idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-ILD) treated at a referral center in Brazil.</p><p><strong>Methods: </strong>This was a retrospective cohort study analyzing demographic characteristics, clinical variables, pulmonary function test results, HRCT findings, serological profiles, treatments, and outcomes.</p><p><strong>Results: </strong>Seventy-nine IIM-ILD patients were included in the present study. The mean follow-up period was 8.7 ± 4.7 years. The most common diagnosis was antisynthetase syndrome, observed in 51 (64.5%) of the 79 patients. The most common symptoms were dyspnea (in 94.9%), arthralgia (in 82.2%), and muscle weakness (in 75.9%). Mean baseline FVC was 2.19 ± 0.75 L, corresponding to 62.5% of the predicted value. During follow-up, FVC showed significant improvement. The most common CT patterns were indeterminate (in 44.4%) and nonspecific interstitial pneumonia (in 35.4%). Treatment most frequently included prednisone (in 98.7%), azathioprine (in 92.3%), or methotrexate (in 57.7%). Overall survival was 84.8%. Mortality was higher among patients who developed pulmonary hypertension and those who required intravenous methylprednisolone pulse therapy.</p><p><strong>Conclusions: </strong>Most patients with IIM-ILD progress well with immunosuppressive therapy. Pulmonary hypertension and the need for methylprednisolone pulse therapy appear to be associated with higher mortality.</p>","PeriodicalId":14845,"journal":{"name":"Jornal Brasileiro De Pneumologia","volume":"51 4","pages":"e20250123"},"PeriodicalIF":3.0,"publicationDate":"2025-10-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12602048/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145421758","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Jornal Brasileiro De Pneumologia
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