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Introduction and aims: There is no systematized information for determining/monitoring the burden of inflammatory bowel disease in Mexico. The aim of the present study was to estimate the annual burden of inflammatory bowel disease on the Mexican National Healthcare System, by number of patients seen, hospitalizations, and specific deaths, stratified into age groups. Materials and methods: Utilizing specific databases of the Mexican National Healthcare System registries coded as ICD-10: K50 and K51, we retrieved and analyzed the data corresponding to the patients seen and hospitalized in 2015, stratified by age group, as well as the specific deaths. Treatment trends among physicians were also examined. Results: In 2015, 5009 women (8.1) and 4944 men (8.4) with Crohn's disease received medical attention (prevalence of cases seen) and 35.1% of those patients were ≥50 years of age. In that same period, 17,177 women (27.7) and 15,883 men (26.9) with ulcerative colitis were seen and 31.6% of those patients were ≥50 years of age. The hospitalized cases (prevalence of hospitalized cases) were 1097 patients (0.91) with Crohn's disease and 43.7% of those patients were ≥50 years of age; and 5345 patients (4.42) with ulcerative colitis and 47.6% of those patients were ≥50 years of age. Deaths (specific mortality rate) were: 32 women (0.52) and 36 men (0.50) due to Crohn's disease, and 267 women (4.31) and 186 men (3.15) due to ulcerative colitis. Conclusions: Inflammatory bowel disease is a burden on the health of Mexican adults and the Mexican National Healthcare System, and it is expected to increase over the next 15 years. Resumen: Introducción y objetivos: En México no existe información sistematizada para determinar/monitorizar la carga de la enfermedad inflamatoria intestinal (EII). El objetivo del estudio fue estimar la carga anual de la EII en el Sistema Nacional de Salud por número de pacientes atendidos, hospitalizaciones y muertes y por grupos de edad. Material y métodos: Utilizando registros específicos de bases de datos del Sistema Nacional de Salud codificados por CIE-10: K50 y K51, obtuvimos y analizamos datos correspondientes a los pacientes atendidos y hospitalizados por grupo etario, así como muertes específicas durante el año 2015. Asimismo, se exploró la tendencia de tratamiento entre médicos. Resultados: En 2015, el número total de casos atendidos (prevalencia de casos atendidos) fue: enfermedad de Crohn en mujeres 5,009 (8.1), en hombres 4,944 (8.4). Los pacientes ≥50 años representaron el 35.1% del total; colitis ulcerosa crónica idiopática en mujeres 17,177 (27.7), en hombres 15,883 (26.9). Los ≥50 años representaron el 31.6% del total. Los casos hospitalizados fueron (prevalencia de casos hospitalizados): enfermedad de Crohn 1,097 (0.91). Los pacientes ≥50 años representaron el 43.7% del total; colitis ulcerosa crónica idiopática 5,345 (4.42). Los enfermos ≥50 años representaron el 47.6% del total. Las defunciones fueron (tasa de muertes específicas): en enfermedad de Crohn: mujeres 32 (0.52), hombres 36 (0.50); colitis ulcerosa crónica idiopática en mujeres 267 (4.31), en hombres 186 (3.15). Conclusiones: La EII representa una carga para la salud de los adultos mexicanos y el Sistema de Salud, y se espera que aumente en los próximos 15 años.

Resumen: Introducción y objetivos: En México no existe información sistematizada para determinar/monitorizar la carga de la enfermedad inflamatoria intestinal (EII). El objetivo del estudio fue estimar la carga anual de la EII en el Sistema Nacional de Salud por número de pacientes atendidos, hospitalizaciones y muertes y por grupos de edad. Material y métodos: Utilizando registros específicos de bases de datos del Sistema Nacional de Salud codificados por CIE-10: K50 y K51, obtuvimos y analizamos datos correspondientes a los pacientes atendidos y hospitalizados por grupo etario, así como muertes específicas durante el año 2015. Asimismo, se exploró la tendencia de tratamiento entre médicos. Resultados: En 2015, el número total de casos atendidos (prevalencia de casos atendidos) fue: enfermedad de Crohn en mujeres 5,009 (8.1), en hombres 4,944 (8.4). Los pacientes ≥ 50 años representaron el 35.1% del total; colitis ulcerosa crónica idiopática en mujeres 17,177 (27.7), en hombres 15,883 (26.9). Los ≥ 50 años representaron el 31.6% del total. Los casos hospitalizados fueron (prevalencia de casos hospitalizados): enfermedad de Crohn 1,097 (0.91). Los pacientes ≥ 50 años representaron el 43.7% del total; colitis ulcerosa crónica idiopática 5,345 (4.42). Los enfermos ≥ 50 años representaron el 47.6% del total. Las defunciones fueron (tasa de muertes específicas): en enfermedad de Crohn: mujeres 32 (0.52), hombres 36 (0.50); colitis ulcerosa crónica idiopática en mujeres 267 (4.31), en hombres 186 (3.15). Conclusiones: La EII representa una carga para la salud de los adultos mexicanos y el Sistema de Salud, y se espera que aumente en los próximos 15 años. Abstract: Introduction and aims: There is no systematized information for determining/monitoring the burden of inflammatory bowel disease in Mexico. The aim of the present study was to estimate the annual burden of inflammatory bowel disease on the Mexican National Healthcare System, by number of patients seen, hospitalizations, and specific deaths, stratified into age groups. Materials and methods: Utilizing specific databases of the Mexican National Healthcare System registries coded as ICD-10: K50 and K51, we retrieved and analyzed the data corresponding to the patients seen and hospitalized in 2015, stratified by age group, as well as the specific deaths. Treatment trends among physicians were also examined. Results: In 2015, 5,009 women (8.1) and 4,944 men (8.4) with Crohn's disease received medical attention (prevalence of cases seen) and 35.1% of those patients were ≥ 50 years of age. In that same period, 17,177 women (27.7) and 15,883 men (26.9) with ulcerative colitis were seen and 31.6% of those patients were ≥ 50 years of age. The hospitalized cases (prevalence of hospitalized cases) were 1,097 patients (0.91) with Crohn's disease and 43.7% of those patients were ≥ 50 years of age; and 5,345 patients (4.42) with ulcerative colitis and 47.6% of those patients were ≥ 50 years of age. Deaths (specific mortality rate) were: 32 women (0.52) and 36 men (0.50) due to Crohn's disease, and 267 women (4.31) and 186 men (3.15) due to ulcerative colitis. Conclusions: Inflammatory bowel disease is a burden on the health of Mexican adults and the Mexican National Healthcare System, and it is expected to increase over the next 15 years.

Background: Interstitial lung disease (ILD) encompasses a heterogeneous group of chronic lung conditions with considerable variability in prognosis and response to treatment. People with reduced muscle mass and function, known as sarcopenia, have a higher risk of mortality and adverse clinical outcomes both in the general population and in other chronic disease states. The importance of sarcopenia across the spectrum of patients with ILD is not well established., Objectives: In this narrative review, we explore the prevalence and clinical implications of sarcopenia in patients with ILD, evaluate the optimal methods to diagnose sarcopenia in this patient population and review treatment interventions., Findings: Almost one third of patients with chronic forms of ILD have evidence of sarcopenia. Sarcopenia is associated with adverse clinical outcomes and increased risk of mortality in select populations with ILD. Screening tests such as the SARC-F (strength, assistance walking, rise from a chair, climb stairs, falls) questionnaire and clinical assessment tools (including grip strength dynamometry) are well validated. Medical imaging modalities, including computed tomography, are hampered by lack of a gold standard and normative values, but have been used in patients with ILD in acute care and research settings. If sarcopenia is identified, multidimensional interventions such as pulmonary rehabilitation are beneficial., Conclusion: Sarcopenia is common in patients with ILD and is associated with poorer outcomes. Accordingly, if identified, targeted interventions should be considered. Validated diagnostic criteria exist, but the optimal use of medical imaging techniques in this patient cohort remains an area of uncertainty., Competing Interests: Conflict of interest: The authors have nothing to disclose., (Copyright ©The authors 2024.)

Background: Lung function testing remains a cornerstone in the assessment and management of interstitial lung disease (ILD) patients. The clinical implications of the Global Lung function Initiative (GLI) reference equations and the updated interpretation strategies remain uncertain., Methods: Adult patients with ILD with baseline forced vital capacity (FVC) were included from the Australasian ILD registry and the National Healthcare Group ILD registry, Singapore.The European Coal and Steel Community and Miller reference equations were compared with the GLI reference equations to assess (a) differences in lung function percent predicted values; (b) ILD risk prediction models and (c) eligibility for ILD clinical trial enrolment., Results: Among 2219 patients with ILD, 1712 (77.2%) were white individuals. Idiopathic pulmonary fibrosis (IPF), connective tissue disease-associated ILD and unclassifiable ILD predominated.Median FVC was 2.60 (2.01-3.36) L, forced expiratory volume in 1 s was 2.09 (1.67-2.66) L and diffusing capacity of the lungs for carbon monoxide (DLCO) was 13.60 (10.16-17.60) mL/min/mm Hg. When applying the GLI reference equations, the mean FVC percentage predicted was 8.8% lower (87.7% vs 78.9%, p<0.01) while the mean DLCO percentage predicted was 4.9% higher (58.5% vs 63.4%, p<0.01). There was a decrease in 19 IPF and 119 non-IPF patients who qualified for the nintedanib clinical trials when the GLI reference equations were applied. Risk prediction models performed similarly in predicting mortality using both reference equations., Conclusion: Applying the GLI reference equations in patients with ILD leads to higher DLCO percentage predicted values and smaller lung volume percentage predicted values. While applying the GLI reference equations did not impact on prognostication, fewer patients met the clinical trial criteria for antifibrotic agents., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: People with interstitial lung disease (ILD) were deemed more vulnerable to the SARS-CoV-2 virus and isolated as a means of reducing risk of infection. This study examined the impact of the pandemic on daily life, psychological wellbeing and access to healthcare and identified approaches undertaken to remain safe., Methods: Four specialist clinics in tertiary centres in Australia (Victoria: two sites; New South Wales: one site; Western Australia: one site) recruited patients with ILD during an 8-week period from March 2021. Semi-structured telephone interviews were conducted with transcripts analysed using principles of grounded theory., Results: Ninety participants were interviewed between April and December 2021. Participants were predominantly female, former smokers with an average age of 66 years. IPF and connective tissue-ILD being the most common subtypes. Five main themes were identified: vulnerability reduced social interaction and isolation, access to healthcare services and support, staying active, emotional and psychological impact. Self-management strategies included staying active both physically and mentally., Discussion: Self-management was key to managing the impact of the pandemic. In combination with advances in technology, implementation of strategies for monitoring wellbeing and support for self-management provides an opportunity to leverage the lessons learnt to ensure a more individualised model of care for people with ILD., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Introduction: The COVID-19 pandemic resulted in a rapid transformation of health services. This study aimed to understand the experiences of healthcare by people with interstitial lung disease (ILD), to inform future service delivery., Methods: Four specialist clinics in tertiary centres in Australia (Victoria:2 sites; New South Wales: 1 site; Western Australia: 1 site) recruited patients with ILD during an 8-week period from March 2021. Participants completed a COVID-specific questionnaire focused on health-related experiences during 2020., Results: Ninety nine (65% of 153) participants completed the questionnaire. 47% had idiopathic pulmonary fibrosis or connective tissue disease-associated ILD, 62% were female and the average age was 66 years. Whilst 56% rated their overall health in 2020 as the same as months prior, 38% indicated a worsening in health attributed to reduced physical activity and fear of contracting the virus. Access to healthcare professionals was 'good' in 61%, and 'fair-to-poor' for 37% due to missed respiratory assessments, with telehealth (mainly telephone) being perceived as less effective. 89% had contact with respiratory physicians, 68% with general practitioners, predominantly via telephone, with few video consultations. High satisfaction with care was reported by 78%, with lower satisfaction attributed to delays in assessments, disruption to usual services such as pulmonary rehabilitation, and dissatisfaction with telehealth., Conclusion: People with ILD were generally satisfied with their care during 2020, however reduced access to healthcare professionals was challenging for those experiencing a deterioration in health. Telehealth was largely well received but did not always meet the needs of people with ILD particularly when unwell., (© 2023. The Author(s).)

Background: The purpose of the National Health and Medical Research Council Centre of Research Excellence in Pulmonary Fibrosis (CRE-PF) is to improve and extend the lives of patients living with pulmonary fibrosis through the development of a comprehensive and integrated program of basic and clinical research and education across Australia. A key objective of the CRE-PF was establishment of a unique national training scheme, CREATE, for early-career researchers (ECRs) in respiratory research. CREATE ECRs are broadly drawn from two main fields of researchers: clinicians and scientists, where clinicians tend to be involved in part-time translational research and scientists are involved in broad scientific research including laboratory or genetic research, health economics or population research., Methods: We describe the CREATE Program which, with limited budget and the assistance of key organisations, has provided funding opportunities (scholarships, fellowships, prizes, travel and collaboration grants), professional development (mentoring program, symposia, presentation opportunities and on-line training) and fostered a connected, supportive research community for respiratory ECRs., Results: The CREATE program has successfully fostered the development of the supported researchers, contributing substantially to the future of pulmonary fibrosis research in Australia. During the life of the program the CRE-PF has offered 10 PhD scholarships and five postdoctoral fellowships, awarded 13 travel grants and three grants to promote collaboration between ECRs from different institutes. A mentoring program has been established and CREATE Symposia have been held in association with key meetings. During COVID-19 restrictions, a series of virtual research meetings has offered 12 CREATE ECRs from seven universities the opportunity to present their research to a national audience. CREATE research-related achievements are impressive, including over 80 first-author publications by ECRs, and many conference presentations. Contributions to the research community, measured by committee membership, is also strong., Conclusions: In spite of a very limited budget, wide geographic distribution of participants and the multi-disciplinary nature of the cohort, we have succeeded in providing a unique, supportive academic development environment for CREATE ECRs. Lessons learned in the process of developing this program include the importance of leveraging funding, being flexible, building networks and seeking and responding to ECR input., (© 2022. The Author(s).)

Background: Interstitial lung disease is a debilitating condition associated with significant dyspnoea, fatigue, and poor exercise tolerance. Pulmonary rehabilitation is an effective and key intervention in people with interstitial lung disease. However, despite the best efforts of patients and clinicians, many of those who participate are not achieving clinically meaningful benefits. This assessor-blinded, multi-centre, randomised controlled trial aims to compare the clinical benefits of high intensity interval exercise training versus the standard pulmonary rehabilitation method of continuous training at moderate intensity in people with fibrotic interstitial lung disease., Methods: Eligible participants will be randomised to either a standard pulmonary rehabilitation group using moderate intensity continuous exercise training or high intensity interval exercise training. Participants in both groups will undertake an 8-week pulmonary rehabilitation program of twice-weekly supervised exercise training including aerobic (cycling) and strengthening exercises. In addition, participants in both groups will be prescribed a home exercise program. Outcomes will be assessed at baseline, upon completion of the intervention and at six months following the intervention by a blinded assessor. The primary outcome is endurance time on a constant work rate test. Secondary outcomes are functional capacity (6-min walk distance), health-related quality of life (Chronic Respiratory Disease Questionnaire (CRQ), St George's Respiratory Questionnaire idiopathic pulmonary fibrosis specific version (SGRQ-I), breathlessness (Dyspnoea 12, Modified Medical Research Council Dyspnoea Scale), fatigue (fatigue severity scale), anxiety (Hospital Anxiety and Depression Scale), physical activity level (GeneActiv), skeletal muscle changes (ultrasonography) and completion and adherence to pulmonary rehabilitation., Discussion: The standard exercise training strategies used in pulmonary rehabilitation may not provide an optimal exercise training stimulus for people with interstitial lung disease. This study will determine whether high intensity interval training can produce equivalent or even superior changes in exercise performance and symptoms. If high intensity interval training proves effective, it will provide an exercise training strategy that can readily be implemented into clinical practice for people with interstitial lung disease. Trial registration ClinicalTrials.gov Registry (NCT03800914). Registered 11 January 2019, https://clinicaltrials.gov/ct2/show/NCT03800914 Australian New Zealand Clinical Trials Registry ACTRN12619000019101. Registered 9 January 2019, https://www.anzctr.org.au/Trial/Registration/TrialReview.aspx?id=376050&isReview=true., (© 2021. The Author(s).)

Aims: Advanced nonmalignant respiratory diseases are associated with significant patient morbidity, yet access to palliative care occurs late, if at all. Our aim was to examine referral criteria for palliative care among patients with advanced nonmalignant respiratory disease, with a view to developing a standardised set of referral criteria., Methods: We performed a systematic review of all studies reporting on referral criteria to palliative care in advanced nonmalignant respiratory disease, with a focus on chronic obstructive pulmonary disease and interstitial lung disease. The systematic review was conducted and reported according to the PRISMA guidelines, and was undertaken using electronic databases (Ovid, MEDLINE, Ovid Embase and PubMed)., Results: Searches yielded 2052 unique titles, which were screened for eligibility and resulted in 62 studies addressing referral criteria to palliative care in advanced nonmalignant respiratory disease. Of 18 categories put forward for referral to palliative care, the most commonly discussed factors were hospital use (69% of papers), indicators of poor respiratory status (47%), physical and emotional symptoms (37%), functional decline (29%), need for advanced respiratory therapies (27%), and disease progression (26%)., Conclusion: Clinicians consider referral to specialist palliative care for a wide range of disease- and needs-based criteria. Our findings highlight the need to standardise palliative care access by developing consensus referral criteria for patients with advanced nonmalignant respiratory illnesses., Competing Interests: Conflict of interest: J. Philip has nothing to disclose. Conflict of interest: A. Collins has nothing to disclose. Conflict of interest: N. Smallwood has nothing to disclose. Conflict of interest: Y.K. Chang has nothing to disclose. Conflict of interest: L. Mo has nothing to disclose. Conflict of interest: I.A. Yang has nothing to disclose. Conflict of interest: T. Corte reports grants from Boehringer Ingelheim, Hoffman-La Roche, Gilead, Biogen, Bayer, Intermune and Actelion, personal fees for consultancy from Boehringer Ingelheim, AstraZeneca, BMS, Promedior and Ad Alta, outside the submitted work. Conflict of interest: C.F. McDonald reports other (fees for lectures paid to institution) from Menarini and AstraZeneca, nonfinancial support (in kind support for research) from Air Liquide, outside the submitted work. Conflict of interest: D. Hui has nothing to disclose., (Copyright ©The authors 2021. For reproduction rights and permissions contact permissions@ersnet.org.)

Structural changes involving tissue remodelling and fibrosis are major features of many pulmonary diseases, including asthma, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). Abnormal deposition of extracellular matrix (ECM) proteins is a key factor in the development of tissue remodelling that results in symptoms and impaired lung function in these diseases. Tissue remodelling in the lungs is complex and differs between compartments. Some pathways are common but tissue remodelling around the airways and in the parenchyma have different morphologies. Hence it is critical to evaluate both common fibrotic pathways and those that are specific to different compartments; thereby expanding the understanding of the pathogenesis of fibrosis and remodelling in the airways and parenchyma in asthma, COPD and IPF with a view to developing therapeutic strategies for each. Here we review the current understanding of remodelling features and underlying mechanisms in these major respiratory diseases. The differences and similarities of remodelling are used to highlight potential common therapeutic targets and strategies. One central pathway in remodelling processes involves transforming growth factor (TGF)-β induced fibroblast activation and myofibroblast differentiation that increases ECM production. The current treatments and clinical trials targeting remodelling are described, as well as potential future directions. These endeavours are indicative of the renewed effort and optimism for drug discovery targeting tissue remodelling and fibrosis., Competing Interests: Declaration of Competing Interest T.C. reports grants and/or personal fees from Boehringer Ingelheim, Roche, Gilead, Bayer, Intermune, AstraZeneca, BMS, Promedior, Ad Alta. C.J.B is a director and shareholder of Amplia Therapeutics, developing anti-fibrotic drugs for the treatment of IPF. P.M.H. has received funding from Pharmaxis for the study of LOX2 inhibitors, and is on the Scientific Advisory Board of Amplia Therapeutics. J.K.B has received research funding from Boehringer Ingelheim. Other authors declare that there are no conflicts of interest., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Interstitial lung diseases are characterised by scarring of lung tissue that leads to reduced transfer of oxygen into the blood, decreased exercise capacity and premature death. Ambulatory oxygen therapy may be used to treat exertional oxyhaemoglobin desaturation, but there is little evidence to support its efficacy and there is wide variation in clinical practice. This study aims to compare the clinical efficacy and cost-effectiveness of ambulatory oxygen versus ambulatory air in people with fibrotic interstitial lung disease and exertional desaturation., Methods and Analysis: A randomised, controlled trial with blinding of participants, clinicians and researchers will be conducted at trial sites in Australia and Sweden. Eligible participants will be randomised 1:1 into two groups. Intervention participants will receive ambulatory oxygen therapy using a portable oxygen concentrator (POC) during daily activities and control participants will use an identical POC modified to deliver air. Outcomes will be assessed at baseline, 3 months and 6 months. The primary outcome is change in physical activity measured by number of steps per day using a physical activity monitor (StepWatch). Secondary outcomes are functional capacity (6-minute walk distance), health-related quality of life (St George Respiratory Questionnaire, EQ-5D-5L and King's Brief Interstitial Lung Disease Questionnaire), breathlessness (Dyspnoea-12), fatigue (Fatigue Severity Scale), anxiety and depression (Hospital Anxiety and Depression Scale), physical activity level (GENEActive), oxygen saturation in daily life, POC usage, and plasma markers of skeletal muscle metabolism, systematic inflammation and oxidative stress. A cost-effectiveness evaluation will also be undertaken., Ethics and Dissemination: Ethical approval has been granted in Australia by Alfred Hospital Human Research Ethics Committee (HREC/18/Alfred/42) with governance approval at all Australian sites, and in Sweden (Lund Dnr: 2019-02963). The results will be published in peer-reviewed scientific journals, presented at conferences and disseminated to consumers in publications for lay audiences., Trial Registration Number: ClinicalTrials.gov Registry (NCT03737409)., Competing Interests: Competing interests: All authors report non-financial support from BOC Australia in the delivery of the trial devices. AEH, YHK, LT, NSLG and CFM report non-financial support from Air Liquide Healthcare, outside the submitted work. YHK reports grants and personal fees from Boehringer Ingelheim, and personal fees from Roche, outside the submitted work. MS received research grants from Boehringer Ingelheim and Roche, outside the submitted work., (© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is an often deadly complication of IPF. No focussed international guidelines for the management of AE-IPF exist. The aim of this international survey was to assess the global variability in prevention, diagnostic and treatment strategies for AE-IPF.Pulmonologists with ILD expertise were invited to participate in a survey designed by an international expert panel.509 pulmonologists from 66 countries responded. Significant geographical variability in approaches to manage AE-IPF was found. Common preventive measures included antifibrotic drugs and vaccination. Diagnostic differences were most pronounced regarding use of Krebs von den Lungen-6 and viral testing, while high-resolution computed tomography, brain natriuretic peptide and D-dimer are generally applied. High-dose steroids are widely administered (94%); the use of other immunosuppressant and treatment strategies is highly variable. Very few (4%) responders never use immunosuppression. Antifibrotic treatments are initiated during AE-IPF by 67%. Invasive ventilation or extracorporeal membrane oxygenation are mainly used as a bridge to transplantation. Most physicians educate patients comprehensively on the severity of AE-IPF (82%) and consider palliative care (64%).Approaches to the prevention, diagnosis and treatment of AE-IPF vary worldwide. Global trials and guidelines to improve the prognosis of AE-IPF are needed., Competing Interests: Conflict of interest: M. Kreuter reports grants and personal fees from Roche, Galapagos and Boehringer, outside the submitted work. Conflict of interest: M. Polke has nothing to disclose. Conflict of interest: S.L.F. Walsh reports personal fees for consultancy from Sanofi-Aventis, Galapagos and OSIC, personal fees for advisory board work from Roche, grants and personal fees for steering committee work from Boehringer Ingelheim, personal fees for lectures from Bracco, outside the submitted work. Conflict of interest: J. Krisam has nothing to disclose. Conflict of interest: H.R. Collard reports personal fees from Bayer, Boehringer Ingelheim, Bristol-Myers Squibb, Global Blood Therapeutics, ImmuneWorks, Navitor, Parexel, Prometic, Toray, Unity, Patara, Veracyte, Roche/Genentech, aTyr, Advance Medical and MedImmune, grants from Pulmonary Fibrosis Foundation, grants and personal fees from Three Lakes Partners, outside the submitted work. Conflict of interest: N. Chaudhuri reports grants from Boehringer Ingelheim and Roche, educational support from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: S. Avdeev has nothing to disclose. Conflict of interest: J. Behr has nothing to disclose. Conflict of interest: G. Calligaro has nothing to disclose. Conflict of interest: T. Corte reports grants, personal fees for lectures and advisory board work, and travel support from Boehringer, grants and personal fees for lectures and advisory board work from Roche, grants from Galapagos, Actelion, Bayer and Sanofi, personal fees for advisory board work from AstraZeneca, outside the submitted work. Conflict of interest: K. Flaherty reports grants and personal fees from Boehringer Ingelheim and Roche/Genentech, personal fees from Bellerophon, Respivant, Veracyte, Sanofi-Genzyme, Blade Therapeutics and Celgene, outside the submitted work. Conflict of interest: M Funke-Chambour has nothing to disclose. Conflict of interest: M. Kolb reports grants and personal fees for consultancy and lectures from Roche and Boehringer Ingelheim, grants and personal fees for consultancy from GSK, Gilead and Prometic, grants from Actelion, Respivert, Alkermes and Pharmaxis, personal fees for consultancy from Genoa, Indalo and Third Pole, outside the submitted work. Conflict of interest: Y. Kondoh reports advisory board fees and lecture fees from Asahi Kasei Pharma Corp. and Boehringer Ingelheim Co., Ltd, advisory board fees from Janssen Pharmaceutical K.K., lecture fees from Eisai Inc., Kyorin Pharmaceutical Co., Ltd, Mitsubishi Tanabe Pharma, Novartis Pharma K.K. and Shionogi and Co., Ltd, outside the present work. Conflict of interest: T.M. Maher has, via his institution, received industry-academic funding from GlaxoSmithKline R&D and UCB, and has received consultancy or speaker fees from Apellis, AstraZeneca, Bayer, Blade Therapeutics, Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos, GlaxoSmithKline R& D, Indalo, Novartis, Pliant, ProMetic, Respivnat, Roche, Samumed and UCB. Conflict of interest: M. Molina Molina reports grants and personal fees from Roche, Boehringer Ingelheim and Esteve-Teijin, grants from GSK and AstraZeneca, personal fees from Pfizer and Chiesi, outside the submitted work. Conflict of interest: A. Morais has nothing to disclose. Conflict of interest: C.C. Moor has nothing to disclose. Conflict of interest: J. Morisset reports personal fees from Hoffmann La Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: C. Pereira has nothing to disclose. Conflict of interest: S. Quadrelli has nothing to disclose. Conflict of interest: M. Selman has nothing to disclose. Conflict of interest: A. Tzouvelekis has nothing to disclose. Conflict of interest: C. Valenzuela reports personal fees for advisory board work and lectures from Roche and Boehringer Ingelheim, personal fees for advisory board work from Galapagos, outside the submitted work. Conflict of interest: C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: V. Vicens-Zygmunt has nothing to disclose. Conflict of interest: J. Wälscher has nothing to disclose. Conflict of interest: W. Wuyts has nothing to disclose. Conflict of interest: M. Wijsenbeek reports grants and fees paid to institution from Boehringer Ingelheim and Hoffman la Roche, fees paid to institution from Galapagos and Novartis, outside the submitted work. Conflict of interest: V. Cottin reports personal fees for advisory board work and lectures, and non-financial (travel) support from Actelion, grants, personal fees for consultancy and lectures, and non-financial (travel) support from Boehringer Ingelheim and Roche, personal fees for advisory board and data monitoring committee work from Bayer/MSD and Galapagos, personal fees for adjudication committee work from Gilead, personal fees for advisory board work and lectures from Novartis, personal fees for lectures from Sanofi, personal fees for data monitoring and steering committee work from Promedior, personal fees for data monitoring committee work from Celgene and Galecto, outside the submitted work. Conflict of interest: E. Bendstrup reports grants and personal fees from Boehringer Ingelheim and Roche, outside the submitted work., (Copyright ©ERS 2020.)

Background: Budesonide has been proven to be an effective treatment for microscopic colitis (MC). However, the two current commercially available preparations are released in the ileum. Beclomethasone dipropionate (Clipper®) is a synthetic corticosteroid with topical colonic release., Objective: This study aimed to explore whether an open-label treatment with beclomethasone dipropionate is an effective treatment for MC., Methods: Prospectively collected data of 30 patients from six centres were retrospectively analysed. All patients had a confirmed diagnosis of idiopathic MC (lymphocytic and collagenous colitis) and were symptomatic (i.e. ≥ 21 loose stools over a seven-day period). Treatment consisted of 10 mg beclomethasone daily for four weeks, followed by 5 mg daily for another four weeks. The primary end point was the proportion of patients in remission (i.e. a mean of < 3 stools/day and a mean of <1 watery stool per day) after an eight-week treatment period. Secondary end points were the proportion of patients responding to therapy at weeks 4 and 8, remission at weeks 4 and 12 and relapse at week 12. Reported adverse events were collected., Results: Overall, at week 8, remission was achieved in 70%, and 77% of patients were responding to treatment. After four weeks of treatment, 80% were responding, and 67% were in remission. Four weeks after stopping treatment, 60% were still in remission., Conclusion: This open-label study suggests that an eight-week course of beclomethasone could be a promising and relatively safe treatment for MC. A randomised controlled study is warranted., (© Author(s) 2019.)

Introduction: Transbronchial lung cryobiopsy (TBLC) is a novel, minimally invasive technique for obtaining lung tissue for histopathological assessment in interstitial lung disease (ILD). Despite its increasing popularity, the diagnostic accuracy of TBLC is not yet known. The COLDICE Study (Cryobiopsy versus Open Lung biopsy in the Diagnosis of Interstitial lung disease allianCE) aims to evaluate the agreement between TBLC and surgical lung biopsy sampled concurrently from the same patients, for both histopathological and multidisciplinary discussion (MDD) diagnoses., Methods and Analysis: This comparative, multicentre, prospective trial is enrolling patients with ILD requiring surgical lung biopsy to aid with their diagnosis. Participants are consented for both video-assisted thoracoscopic surgical (VATS) biopsy and TBLC within the same anaesthetic episode. Specimens will be blindly assessed by three expert pathologists both individually and by consensus. Each tissue sample will then be considered in conjunction with clinical and radiological data, within a centralised MDD. Each patient will be presented twice in random order, once with TBLC data and once with VATS data. Meeting participants will be blinded to the method of tissue sampling. The accuracy of TBLC will be assessed by agreement with VATS at (1) histopathological analysis and (2) MDD diagnosis. Data will be collected on interobserver agreement between pathologists, interobserver agreement between MDD participants, and detailed clinical and procedural characteristics., Ethics and Dissemination: The study is being conducted in accordance with the International Conference on Harmonisation Guideline for Good Clinical Practice and Australian legislation for the ethical conduct of research., Trial Registration Number: ACTRN12615000718549., Competing Interests: Competing interests: LKT received study-related unrestricted educational grants or inkind support from the aforementioned commercial entities, on behalf of the COLDICE Investigator Team. MPV sits on the advisory boards of Medtronic and Edwards Lifesciences, and consults for Edwards Lifesciences and Abbott. GR provides consultation for Bellerophon, Biogen, BMS, FibroGen, Gilead, Nitto, Revistan, Promedior, Sanofi, Veracyte, Roche-Genentech, Avalyn and Boehringer Ingelheim. JM is an unpaid collaborator in the Veracyte BRAVE Trial.

Background: In Australia, government-subsidised treatment of pulmonary arterial hypertension (PAH) is limited to monotherapy. Recent international guidelines advocate that initial combination therapy be considered for all symptomatic PAH patients., Aim: To characterise 'real-life' outcomes in PAH patients initiated on monotherapy., Methods: We performed a retrospective analysis of 100 consecutive PAH patients at a single centre who were commenced on monotherapy for PAH between 2004 and 2015. The composite clinical end-point of 'treatment failure' was prospectively defined as (i) >15% fall in 6-min walk distance (6MWD) on follow up, (ii) physician judgement of inadequate treatment response, (iii) adverse drug effect requiring cessation and (iv) death or transplantation., Results: At initiation of therapy, mean age was 54 ± 18 years, and underlying diagnoses included idiopathic (36%), connective tissue disease-associated (37%) and congenital heart disease-associated-PAH (25%). Baseline 6MWD was 360 ± 140 m, and 75% were in either the New York Heart Association functional classes III or IV. Over a median follow up of 38 months (interquartile range 20-67), 62% of the subjects met the criteria for a clinical failure event. Median time to monotherapy failure was 24 months (95% confidence interval 14-34), with death or transplantation being the most common clinical failure event. Estimated 1-, 3- and 5-year survival rates from time of treatment initiation were 92, 75 and 66%., Conclusion: The majority of patients failed initial monotherapy therapy within 2 years of treatment initiation. Broader access to approved PAH agents is needed to enable combination therapy in line with evidence-based international guidelines., (© 2017 Royal Australasian College of Physicians.)

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive disease characterised by dyspnea and loss of lung function., Methods: Using pooled data from the replicate, randomized, 52-week, placebo-controlled INPULSIS(®) trials, we characterized the safety and tolerability of nintedanib 150 mg twice daily in patients with IPF and described how adverse events were managed during these trials., Results: One thousand and sixty- one patients were treated (nintedanib 638; placebo 423). Higher proportions of patients in the nintedanib group than the placebo group had ≥ 1 dose reduction to 100 mg bid (27.9% versus 3.8%) or treatment interruption (23.7% versus 9.9%). Adverse events led to permanent treatment discontinuation in 19.3% and 13.0% of patients in the nintedanib and placebo groups, respectively. Diarrhea was the most frequent adverse event, reported in 62.4% of patients in the nintedanib group versus 18.4% in the placebo group; however, only 4.4% of nintedanib-treated patients discontinued trial medication prematurely due to diarrhea. Monitoring of liver enzymes before and periodically during nintedanib treatment was recommended so that liver enzyme elevations could be managed through dose reduction or treatment interruption., Conclusion: Nintedanib had a manageable safety and tolerability profile in patients with IPF. Recommendations for adverse event management minimized permanent treatment discontinuations in the INPULSIS(®) trials., Trial Registration: clinicaltrials.gov NCT01335464 and NCT01335477.

Background: Interstitial lung disease (ILD) includes a diverse group of respiratory conditions characterised by inflammation and fibrosis of the interstitium. Worsening hypoxia and respiratory failure may develop with disease progression. Disease behaviour and treatment responsiveness vary widely depending on the underlying aetiology. One of the most common types of ILD is idiopathic pulmonary fibrosis (IPF), which is associated with poor prognosis., Objective: This article discusses recent advances in the field of ILD, including updated classification, diagnostic approach and break-through therapies., Discussion: Establishing an accurate diagnosis in patients with ILD, ideally within a multidisciplinary team discussion, is critical for ensuring optimal outcomes. Recently, novel antifibrotic therapies have been shown to be effective in slowing disease progression in IPF, offering new hope for patients with the disease.

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population., (© 2015 Asian Pacific Society of Respirology.)

The idiopathic inflammatory myopathies, including dermatomyositis, are uncommon acquired autoimmune diseases, sometimes associated with interstitial lung disease. Myasthenia gravis, a separate autoimmune disorder involving the neuromuscular junction, has some overlapping clinical features but has only rarely been reported to occur simultaneously within the same patient. Here we present the first reported case of concomitant dermatomyositis, myasthenia gravis, and interstitial lung disease.

Objectives: In a multi-centre study, we sought to determine whether extent of disease on high-resolution CT (HRCT) lung, reported using a simple grading system, is predictive of decline and mortality in SSc-related interstitial lung disease (SSc-ILD), independently of pulmonary function tests (PFTs) and other prognostic variables., Methods: SSc patients with a baseline HRCT performed at the time of ILD diagnosis were identified. All HRCTs and PFTs performed during follow-up were retrieved. Demographic and disease-related data were prospectively collected. HRCTs were graded according to the percentage of lung disease: >20%: extensive; <20%: limited; unclear: indeterminate. Indeterminate HRCTs were converted to limited or extensive using a forced vital capacity threshold of 70%. The composite outcome variable was deterioration (need for home oxygen or lung transplantation), or death., Results: Among 172 patients followed for mean (s.d.) of 3.5 (2.9) years, there were 30 outcome events. In Weibull multivariable hazards regression modelling, baseline HRCT grade was independently predictive of outcome, with an adjusted hazard ratio (aHR) = 3.0, 95% CI 1.2, 7.5 and P = 0.02. In time-varying covariate models (based on 1309 serial PFTs and 353 serial HRCTs in 172 patients), serial diffusing capacity of the lung for carbon monoxide by alveolar volume ratio (ml/min/mmHg/l) (aHR = 0.4; 95% CI 0.3, 0.7; P = 0.001) and forced vital capacity (dl) (aHR = 0.9; 95% CI 0.8, 0.97; P = 0.008), were also strongly predictive of outcome., Conclusion: Extensive disease (>20%) on HRCT at baseline, reported using a semi-quantitative grading system, is associated with a three-fold increased risk of deterioration or death in SSc-ILD, compared with limited disease. Serial PFTs are informative in follow-up of patients.

We evaluated the handling performance at implant, and the long-term atrial and ventricular electrical performance of a new generation using a very small surface area (1.2 mm2) steroid-eluting electrode (Medtronic CapSure Z). We compared the performance of CapSure Z to that of traditional passive fixation leads, with and without steroid elution. The study was conducted during 2 years of follow-up. We studied 188 patients (105 males and 83 females; mean age 71 +/- 7 years). All of the patients were implanted with a dual chamber pacemaker and the same type of lead in both chambers. Forty-one patients received CapSure Z leads, 25 patients received Target Tip leads (8-mm2 surface area; no steroid elution), 63 patients received CapSure leads (8-mm2 surface area; steroid elution), and 59 patients received CapSure SP leads (5.8-mm2 surface area; steroid elution). The four groups were homogeneous in regards to sex, age, cardiac disease, and reason for implant. At follow-up, the CapSure Z lead showed sensing values comparable to the other leads, with lower pacing thresholds and higher pacing impedance in both chambers. We evaluated the mean current drained from the pacemaker by the different types of leads when using safe, low energy output settings. We found that by using CapSure Z leads, the mean current was significantly lower than that of the other types of leads (0.42 microA for CapSure Z ventricular lead vs 0.85 for CapSure SP, 1.42 for CapSure, and 1.54 for Target Tip). Thus, the use of the CapSure Z lead, combined with low energy output programming, will increase pacemaker longevity compared to the use of traditional leads and standard output programming.