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Skin side-effects of elexacaftor-tezacaftor-ivacaftor: a real-world view 皮肤副作用:一个真实世界的观点。
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-10-27 DOI: 10.1016/j.resmer.2025.101218
Sophie Constantinou , Lucy Webber , Alice Darby , Devika Sunil , Mirain Phillips , Richard Goodwin , Jamie Duckers

Background

Since 2020, ETI (elexacaftor/tezacaftor/ivacaftor) has been used to treat patients with cystic fibrosis. Acne and rash are listed as “common” and “very common” adverse effects in the Summary of Product Characteristics for ETI, affecting between 2-5% and 10% of patients respectively. Patients have, however, shared their experience of developing acne and rashes with clinicians and on social media. This study aimed to describe the real-world impact of rash and acne amongst patients taking ETI and to evaluate the service provided by healthcare practitioners.

Methods

A paper and/or electronic survey was distributed to adult patients taking ETI in two regional CF centres between November 2022 and February 2023.

Results

Of the 268 survey respondents, 20.9% reported new-onset acne and 13.8% reported a rash following the commencement of ETI. 11.9% had a past history of acne and reported an exacerbation/recurrence after starting ETI. ETI-associated acne occurred weeks to months after commencement of ETI, whereas most rashes occurred within the first 2 weeks of treatment. Reassuringly for 2/3 of affected patients, acne and rashes were minor side effects, although 1/3 experienced a greater impact on their quality of life.

Conclusion

This study is the first to specifically report the real-world prevalence of acne and rashes amongst patients taking ETI, which is higher than that reported in clinical trials. These findings could be used to improve counselling of patients commencing ETI. Ensuring access to up-to-date resources and treatments for patients experiencing skin side-effects would be mutually beneficial for both patients and healthcare professionals.
背景:自2020年以来,ETI (elexacaftor/tezacaftor/ivacaftor)已被用于治疗囊性纤维化患者。痤疮和皮疹在ETI产品特性摘要中被列为“常见”和“非常常见”的不良反应,分别影响2-5%和10%的患者。然而,患者与临床医生和社交媒体分享了他们患痤疮和皮疹的经历。本研究旨在描述在接受ETI的患者中皮疹和痤疮的现实影响,并评估医疗保健从业者提供的服务。方法:在2022年11月至2023年2月期间,向两个地区CF中心接受ETI的成年患者分发了一份论文和/或电子调查。结果:在268名受访者中,20.9%报告了新发痤疮,13.8%报告了ETI开始后的皮疹。11.9%的患者既往有痤疮病史,并在开始ETI后出现恶化/复发。与ETI相关的痤疮发生在ETI开始后的几周到几个月,而大多数皮疹发生在治疗的前两周。令人欣慰的是,对于2/3的受影响患者来说,痤疮和皮疹是轻微的副作用,尽管1/3的患者的生活质量受到了更大的影响。结论:本研究首次明确报道了服用ETI的患者中痤疮和皮疹的真实患病率,高于临床试验报告。这些发现可用于改善对开始ETI的患者的咨询。确保有皮肤副作用的患者获得最新的资源和治疗,对患者和医疗保健专业人员都是有利的。
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引用次数: 0
Addressing the inequities in interstitial lung disease care outside specialised centres in Australia 解决澳大利亚专业中心以外间质性肺病护理方面的不平等问题
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-10-08 DOI: 10.1016/j.resmer.2025.101217
Gabriella Tikellis , Anne E Holland
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引用次数: 0
Corrigendum to “Particle size distribution of viable nebulized bacteriophage for the treatment of multi-drug resistant Pseudomonas aeruginosa” [Respiratory Medicine and Research 86 (2024) 101133] “治疗多重耐药铜绿假单胞菌的活菌雾化噬菌体粒径分布”[呼吸医学与研究86(2024)101133]的勘误。
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-10-08 DOI: 10.1016/j.resmer.2025.101209
Daniel L Thompson , Zoe Semersky , Richard Feinn , Pamela Huang , Paul E Turner , Ben K Chan , Jonathan L Koff , Thomas S Murray
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引用次数: 0
First steps for telemonitoring mechanical cough assistance in people with neuromuscular diseases: The Tele-INEX study 神经肌肉疾病患者远程监测机械咳嗽辅助的第一步:Tele-INEX研究
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-10-03 DOI: 10.1016/j.resmer.2025.101213
M Lebret , D Zerillo , A Kerfourn , M Mahot , R Abouly , E Fresnel , JC Borel
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引用次数: 0
The predicted value of maximal inspiratory pressure, maximal expiratory pressure, and sniff nasal inspiratory pressure for southeast asian adults 东南亚成人最大吸气压力、最大呼气压力和嗅鼻吸气压力的预测值
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-30 DOI: 10.1016/j.resmer.2025.101212
Ngoc-Minh Nguyen , Hanh Thi-Bich Tran , Thi-Quynh-Nhu Do , Nicolas Audag , Giuseppe Liistro , Philippe Fait , Gregory Reychler

Background

Accurate assessment of respiratory muscle strength is crucial for diagnosing and managing respiratory diseases. However, existing reference values may not be generalizable across diverse populations. This study aimed to establish predicted values for maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP) in Southeast Asian adults.

Methods

MIP, MEP, and SNIP were measured in 301 healthy Vietnamese adults. Two-way ANOVA and post-hoc tests were used to examine differences in these measures among age groups and between genders. Stepwise multiple linear regression was used to develop predictive equations for MIP, MEP, and SNIP, with potential predictors including age, gender, body mass index, and lung function. The lower limit of the normal range (LLN) was determined using the fifth percentile of the negative residuals.

Results

MIP, MEP, and SNIP were higher in males than in females. MIP and MEP declined with age, while SNIP remained relatively stable. Predictive equations were established: MIP = 95.2 – 32.1 x gender (male = 0, female = 1) – 0.41 x age + 1.2 x BMI (adjusted R2: 41 %, LLN = predicted MIP – 34), MEP = 135.1 – 46.75 x gender (male = 0, female = 1) - 0.6 x age + 1.34 x BMI (adjusted R2: 41.8 %, LLN = predicted MEP – 50), SNIP = 63.8 – 18.16 x gender (male = 0, female = 1) (adjusted R2: 14.8 %, LLN = predicted SNIP – 30).

Conclusions

This study provides ethnic-specific predictive equations for MIP, MEP, and SNIP, which may serve as a preliminary step toward developing reference values for the Southeast Asian region.
背景:准确评估呼吸肌力量对诊断和治疗呼吸系统疾病至关重要。然而,现有的参考值可能无法在不同的人群中推广。本研究旨在建立东南亚成年人最大吸气压力(MIP)、最大呼气压力(MEP)和嗅鼻吸气压力(SNIP)的预测值。方法测定301例越南健康成人的smip、MEP和SNIP。采用双向方差分析和事后检验来检验这些措施在年龄组和性别之间的差异。采用逐步多元线性回归建立MIP、MEP和SNIP的预测方程,潜在的预测因子包括年龄、性别、体重指数和肺功能。正常范围的下限(LLN)是用负残差的第5个百分位数确定的。结果男性smip、MEP、SNIP均高于女性。MIP和MEP随年龄增长而下降,而SNIP保持相对稳定。建立了预测方程:MIP = 95.2 - 32.1 x性别(男= 0,女= 1)- 0.41 x + 1.2岁BMI (R2调整:41%,LLN =预测MIP - 34),欧洲议会议员= 135.1 - 46.75 x性别(男= 0,女= 1)- 0.6 x + 1.34岁BMI (R2调整:41.8%,LLN =预测MEP - 50),剪断= 63.8 - 18.16 x性别(男= 0,女= 1)(R2调整:14.8%,LLN =预测剪断- 30)。结论本研究提供了种族特异性的MIP、MEP和SNIP预测方程,为东南亚地区开发参考值奠定了基础。
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引用次数: 0
When to test for myositis antibodies in usual interstitial pneumonia on chest CT? 在常规间质性肺炎的胸部CT上什么时候检测肌炎抗体?
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-26 DOI: 10.1016/j.resmer.2025.101211
Eliott Guillois , Daniel Bertin , Jules Milesi , Paul Habert , Mathieu Di Bisceglie , Xavier Heim , Benjamin Coiffard , Romain Naud , Ana Nieves , Ngoc Anh Thu Nguyen , Martine Reynaud-Gaubert , Nathalie Bardin , Julien Bermudez

Introduction

Usual interstitial pneumonia (UIP) is a key pattern of interstitial lung disease (ILD), most commonly linked to idiopathic pulmonary fibrosis (IPF). Identifying underlying autoimmune conditions such as myositis is clinically relevant, yet guidelines provide limited recommendations regarding myositis antibody (MSA) screening.

Methods

We retrospectively analyzed patients with UIP who underwent systematic MSA screening at our center. Clinical and serological characteristics were compared between MSA-positive (MSA+) and MSA-negative (MSA-) groups. Logistic regression was used to identify predictive factors.

Results

Among 134 patients, 15 (11 %) were MSA+. Compared with MSA- patients, MSA+ cases were more likely to present with autoimmune disease (p = 0.03), ANA ≥ 1:320 (p < 0.001), and positive rheumatoid factor (p = 0.04). A four-parameter profile combining ANA < 1:320, absence of rheumatoid factor, no hypergammaglobulinemia, and no Raynaud’s phenomenon strongly predicted MSA negativity with excellent specificity (100 %), modest sensitivity (35 %), and good overall discriminative ability (AUC = 0.82).

Discussion

In patients with UIP, a simple four-parameter profile (ANA < 1:320, absence of rheumatoid factor, absence of hypergammaglobulinemia, and absence of Raynaud’s phenomenon) strongly predicts negative MSA status. These findings support a more targeted approach to MSA screening, potentially improving diagnostic accuracy, guiding treatment decisions, and reducing unnecessary testing in UIP.
常见性间质性肺炎(UIP)是间质性肺病(ILD)的一种关键类型,最常与特发性肺纤维化(IPF)相关。识别潜在的自身免疫性疾病如肌炎具有临床相关性,但指南对肌炎抗体(MSA)筛查的建议有限。方法回顾性分析在本中心接受系统MSA筛查的UIP患者。比较MSA阳性(MSA+)组和MSA阴性(MSA-)组的临床和血清学特征。采用Logistic回归分析确定预测因素。结果134例患者中MSA+ 15例(11%)。与MSA-患者相比,MSA+患者更容易出现自身免疫性疾病(p = 0.03), ANA≥1:20 20 (p < 0.001),类风湿因子阳性(p = 0.04)。ANA < 1:20 20,无类风湿因子,无高γ球蛋白血症,无雷诺现象的四参数谱能预测MSA阴性,具有良好的特异性(100%),适度的敏感性(35%)和良好的总体判别能力(AUC = 0.82)。在UIP患者中,简单的四参数谱(ANA < 1:320,无类风湿因子,无高γ球蛋白血症,无雷诺现象)强有力地预测了MSA的阴性状态。这些发现支持一种更有针对性的MSA筛查方法,可能提高诊断准确性,指导治疗决策,并减少UIP中不必要的检测。
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引用次数: 0
Acute hemodynamic response to inhaled treprostinil in pulmonary hypertension associated with interstitial lung disease: a case highlighting unresolved questions 肺高血压合并间质性肺疾病患者吸入曲前列替尼的急性血流动力学反应:一个突出未解决问题的病例
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-25 DOI: 10.1016/j.resmer.2025.101210
Marianne Riou , Matthieu Canuet , Sandrine Hirschi , Ségolène Turquier , David Montani
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引用次数: 0
MUC5B polymorphism and post COVID-19 lung abnormalities on chest CT-scan MUC5B多态性与胸部ct扫描新冠肺炎后肺部异常
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-17 DOI: 10.1016/j.resmer.2025.101207
Celestie Yaacoub , Coralie Tardivon , Jean Dib , Spyridon Prountzos , Joe Yazbeck , Elvira-Markela Antonogiannaki , Ibrahima Ba , Christina Kontopoulou , Pinelopi Kazakou , Lykourgos Kolilekas , Anastasia Antoniadou , Bruno Crestani , Spyros A. Papiris , Effrosyni D. Manali , Quentin Philippot , Antoine Khalil , Cedric Laouenan , Raphael Borie , Marie-Pierre Debray , on behalf of the French COVID cohort study group
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引用次数: 0
Understanding participation challenges in lung cancer screening program: Findings from the DEP’KP80 trial in France 了解参与肺癌筛查项目的挑战:来自法国DEP 'KP80试验的发现
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-17 DOI: 10.1016/j.resmer.2025.101208
Sébastien Couraud , Emmanuel Grolleau , Bernard Milleron , Valérie Petigny , Olivier Leleu

Background

Lung cancer is the leading cause of cancer-related mortality worldwide. Low-dose computed tomography (LDCT) screening can significantly reduce mortality among high-risk populations. However, participation rates in lung cancer screening programs are often suboptimal due to various barriers.

Method

The DEP’KP80 trial is a multicentric prospective study conducted in the French territory of Somme, assessing the feasibility of lung cancer screening using LDCT. Participants aged 55–74 with a smoking history of over 30 pack-years were recruited between 2016 and 2020. A questionnaire was administered to all participants to evaluate motivations and barriers to participation.

Results

The trial enrolled 1369 participants, with 30.4 % responding to the questionnaire. Of the responders, 65 % were male, and the mean age was 61.9 years. Key motivations for participation included health concerns related to smoking (68.7 %) and healthcare professional advice (16.5 %). Main barriers were perceived lack of invitation (37.5 %) and logistical issues (21.9 %). Notably, 63 % of responders reported that participation led to smoking cessation. In addition, the most common source of information about the program was healthcare professionals (85 %).

Conclusion

Our study highlights the crucial role of healthcare professionals in promoting lung cancer screening and identifies significant barriers to participation. Addressing these barriers through targeted interventions could enhance screening uptake and improve lung cancer outcomes.
背景肺癌是全球癌症相关死亡的主要原因。低剂量计算机断层扫描(LDCT)筛查可显著降低高危人群的死亡率。然而,由于各种障碍,肺癌筛查项目的参与率往往不是最佳的。DEP’kp80试验是一项在法国索姆省开展的多中心前瞻性研究,旨在评估LDCT筛查肺癌的可行性。参与者年龄在55-74岁之间,吸烟史超过30包年,于2016年至2020年间招募。对所有参与者进行了问卷调查,以评估参与的动机和障碍。结果共纳入1369名受试者,30.4 %的受试者回答了问卷调查。在应答者中,65% %为男性,平均年龄为61.9岁。参与的主要动机包括与吸烟有关的健康问题(68.7% %)和保健专业咨询(16.5% %)。主要障碍是缺乏邀请(37.5% %)和后勤问题(21.9% %)。值得注意的是,63% %的应答者报告说参与导致了戒烟。此外,关于该计划的最常见信息来源是医疗保健专业人员(85% %)。结论我们的研究强调了医疗保健专业人员在促进肺癌筛查方面的关键作用,并确定了参与筛查的重大障碍。通过有针对性的干预措施解决这些障碍,可以提高筛查的接受程度,改善肺癌的预后。
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引用次数: 0
Classification of systemic lupus erythematosus in patients with interstitial pneumonia with autoimmune features using two different sets of criteria 两套不同标准对具有自身免疫特征的间质性肺炎患者系统性红斑狼疮的分类
IF 1.8 4区 医学 Q3 RESPIRATORY SYSTEM Pub Date : 2025-09-17 DOI: 10.1016/j.resmer.2025.101206
Gabriela Martinez-Zayas , David Karp , Traci N. Adams , Elena K. Joerns

Objective

Interstitial pneumonia with autoimmune features (IPAF) describes patients with interstitial lung disease and autoimmunity who do not meet classification criteria for a systemic autoimmune rheumatic disease, including systemic lupus erythematosus (SLE). We aimed to determine whether the criteria developed by the European Alliance of Association of Rheumatology and American College of Rheumatology (EULAR/ACR) would classify more patients with IPAF as SLE, than those developed by the Systemic Lupus International Collaborating Clinics (SLICC). Increasing the recognition of SLE among patients with IPAF may affect therapy.

Methods

This observational, retrospective, single-center cohort study included consecutive patients initially classified as having IPAF between December 2005-August 2019. We reviewed patients’ charts to assess whether more patients met SLE criteria by SLICC or EULAR/ACR methodology. Fisher’s exact test assessed the significance of the difference in the proportion of patients classified by the two criteria.

Results

We included 201 patients initially classified as IPAF. Twelve were identified as SLE by SLICC criteria and 23 by EULAR/ACR criteria. All but three patients with lymphopenia who met SLE criteria by SLICC also met it by EULAR/ACR. The difference in the proportion of IPAF patients meeting SLE criteria by the two methods was statistically significant (p < 0.001).

Conclusions

Patients with IPAF were classified as SLE more frequently using EULAR/ACR criteria than by SLICC criteria. The EULAR/ACR criteria may be superior for SLE classification in patients with ILD. Patients with IPAF and SLE features should undergo full work-up to evaluate for SLE classification as it may affect therapy.
目的:具有自身免疫性特征的间质性肺炎(IPAF)描述了不符合系统性自身免疫性风湿病(包括系统性红斑狼疮(SLE))分类标准的间质性肺疾病和自身免疫患者。我们的目的是确定由欧洲风湿病协会联盟和美国风湿病学会(EULAR/ACR)制定的标准是否会比由系统性狼疮国际合作诊所(SLICC)制定的标准将更多的IPAF患者分类为SLE。提高IPAF患者对SLE的认识可能会影响治疗。方法:这项观察性、回顾性、单中心队列研究纳入了2005年12月至2019年8月期间最初被分类为IPAF的连续患者。我们回顾了患者的图表,以评估是否有更多的患者符合SLICC或EULAR/ACR方法的SLE标准。Fisher的精确检验评估了按两种标准分类的患者比例差异的显著性。结果:我们纳入了201例初始分类为IPAF的患者。12例根据SLICC标准确诊为SLE, 23例根据EULAR/ACR标准确诊为SLE。除3例淋巴细胞减少患者外,所有通过SLICC达到SLE标准的患者也通过EULAR/ACR达到SLE标准。两种方法IPAF患者符合SLE标准的比例差异有统计学意义(p < 0.001)。结论:与SLICC标准相比,EULAR/ACR标准更频繁地将IPAF患者分类为SLE。对于ILD患者的SLE分类,EULAR/ACR标准可能更优。具有IPAF和SLE特征的患者应进行全面检查以评估SLE的分类,因为它可能影响治疗。
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引用次数: 0
期刊
Respiratory Medicine and Research
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