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Background: Pulmonary alveolar proteinosis (PAP) is a rare syndrome caused by several distinct diseases leading to progressive dyspnoea, hypoxaemia, risk of respiratory failure and early death due to accumulation of proteinaceous material in the lungs. Diagnostic strategies may include computed tomography (CT) of the lungs, bronchoalveolar lavage (BAL), evaluation of antibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF), genetic testing and, eventually, lung biopsy. The management options are focused on removing the proteinaceous material by whole lung lavage (WLL), augmentation therapy with GM-CSF, rituximab, plasmapheresis and lung transplantation. The presented diagnostic and management guidelines aim to provide guidance to physicians managing patients with PAP., Methods: A European Respiratory Society Task Force composed of clinicians, methodologists and patients with experience in PAP developed recommendations in accordance with the ERS Handbook for Clinical Practice Guidelines and the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) approach. This included a systematic review of the literature and application of the GRADE approach to assess the certainty of evidence and strength of recommendations. The Task Force formulated five PICO (Patients, Intervention, Comparison, Outcomes) questions and two narrative questions to develop specific evidence-based recommendations., Results: The Task Force developed recommendations for the five PICO questions. These included management of PAP with WLL, GM-CSF augmentation therapy, rituximab, plasmapheresis and lung transplantation. Also, the Task Force made recommendations regarding the use of GM-CSF antibody testing, diagnostic BAL and biopsy based on the narrative questions. In addition to the recommendations, the Task Force provided information on the hierarchy of diagnostic interventions and therapy., Conclusions: The diagnosis of PAP is based on CT and BAL cytology or lung histology, whereas the diagnosis of specific PAP-causing diseases requires GM-CSF antibody testing or genetic analysis. There are several therapies including WLL and augmentation therapy with GM-CSF available to treat PAP, but supporting evidence is still limited., Competing Interests: Conflict of interest: C. McCarthy, F. Bonella, B.C. Trapnell and M. Griese report membership of a scientific advisory board of Savara Inc. B.C. Trapnell reports grants from the NHLBI (HL085453) and Savara, consultancy fees from Savara, and support for attending meetings from Savara. T. Wang reports grants from Savara, consultancy fees from Partner Therapeutics and Savara, payment or honoraria for lectures, presentations, manuscript writing or educational events from Partner Therapeutics and Savara, support for attending meetings from the PAP Foundation, participation on a data safety monitoring board or advisory board with Partner Therapeutics and Savara Inc., and a leadership role with the PAP Foundation (Clinical Director and Vice President). C. McCarthy reports grants from Health Research Board, Ireland, Enterprise Ireland and The LAM Foundation, consultancy fees from Theravance Inc., Savara Inc. and AI Therapeutics, support for attending meetings from Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Savara Inc. F. Bonella reports consultancy fees from Boehringer Ingelheim, Sanofi, BMS and Savara Inc., payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Sanofi, support for attending meetings from Boehringer Ingelheim, AstraZeneca, Atyr and Savara Pharma, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Sanofi and BMS. M. Fally reports leadership roles with the European Respiratory Society (Member of the Clinical Practice Guidelines Methodology Network and Secretary of Assembly 10, Group 1) and Danish Medical Journal (Associate Editor). A. Hadchouel reports patent issued (PCT/EP2022/064179). S. Jouneau reports the following financial (or non-financial) interests: PI for IMPALA and IMPALA-2 studies (Savara Inc.). R. Borie reports honoraria from Boehringer Ingelheim, Sanofi and Ferrer, support for attending meetings from Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Savara. I. Campo reports consultancy fees from Partner's Therapeutics, and participation on a data safety monitoring board or advisory board with Savara. E. Manali reports grants from Savara, consulting fees from Boehringer Ingelheim, CLS Behring and Hoffman-La Roche, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, CLS Behring and Hoffman-La Roche, support for attending meetings from Boehringer Ingelheim, CLS Behring, Hoffman-La Roche and Elpen, and a leadership role with the European Respiratory Society (Chair in ERS Task Force for Transition of chILD). H. Prosch reports grants from Boehringer Ingelheim, AstraZeneca, Siemens Healthineers, the Christian Doppler Research Association and EU Commission (EU4Health, Horizon Europe Health), consultancy fees from Boehringer Ingelheim and Sanofi, payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, BMS, Boehringer Ingelheim, Bracco, Daiichi Sankyo, Janssen, MSD, Novartis, Roche, Sanofi, Siemens Healthineers and Takeda, support for attending meetings from Boehringer Ingelheim, participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, and a leadership role with the European Society of Thoracic Imaging (Vice President). M. Veltkamp reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Chiesi, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim and Xentria. E. Bendstrup reports honoraria from Boehringer Ingelheim, AstraZeneca and Daichii Sankyo, support for attending meetings from Boehringer Ingelheim, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim and Simbec-Orion. The remaining authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024. For reproduction rights and permissions contact permissions@ersnet.org.)

Pulmonary alveolar proteinosis (PAP) is a syndrome that results from the accumulation of lipoproteinaceous material in the alveolar space. According to the underlying pathogenetic mechanisms, three different forms have been identified, namely primary, secondary and congenital. Primary PAP is caused by disruption of granulocyte-macrophage colony-stimulating factor (GM-CSF) signalling due to the presence of neutralising autoantibodies (autoimmune PAP) or GM-CSF receptor genetic defects (hereditary PAP), which results in dysfunctional alveolar macrophages with reduced phagocytic clearance of particles, cholesterol and surfactant. The serum level of GM-CSF autoantibody is the only disease-specific biomarker of autoimmune PAP, although it does not correlate with disease severity. In PAP patients with normal serum GM-CSF autoantibody levels, elevated serum GM-CSF levels is highly suspicious for hereditary PAP. Several biomarkers have been correlated with disease severity, although they are not specific for PAP. These include lactate dehydrogenase, cytokeratin 19 fragment 21.1, carcinoembryonic antigen, neuron-specific enolase, surfactant proteins, Krebs von Lungen 6, chitinase-3-like protein 1 and monocyte chemotactic proteins. Finally, increased awareness of the disease mechanisms has led to the development of pathogenesis-based treatments, such as GM-CSF augmentation and cholesterol-targeting therapies., Competing Interests: Conflict of interest: F. Bonella reports consultancy fees from Boehringer Ingelheim, Sanofi, BMS and CSL-Behring, payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Sanofi, support for attending meetings from Boehringer Ingelheim, Astra Zeneca and Atyr, and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Sanofi and BMS. I. Campo reports consultancy fees from Partner Therapeutics, and participation on a data safety monitoring board or advisory board with Savara. All other authors have nothing to disclose., (Copyright ©The authors 2024.)

Genetic predisposition to pulmonary fibrosis has been confirmed by the discovery of several gene mutations that cause pulmonary fibrosis. Although genetic sequencing of familial pulmonary fibrosis (FPF) cases is embedded in routine clinical practice in several countries, many centres have yet to incorporate genetic sequencing within interstitial lung disease (ILD) services and proper international consensus has not yet been established. An international and multidisciplinary expert Task Force (pulmonologists, geneticists, paediatrician, pathologist, genetic counsellor, patient representative and librarian) reviewed the literature between 1945 and 2022, and reached consensus for all of the following questions: 1) Which patients may benefit from genetic sequencing and clinical counselling? 2) What is known of the natural history of FPF? 3) Which genes are usually tested? 4) What is the evidence for telomere length measurement? 5) What is the role of common genetic variants (polymorphisms) in the diagnostic workup? 6) What are the optimal treatment options for FPF? 7) Which family members are eligible for genetic sequencing? 8) Which clinical screening and follow-up parameters may be considered in family members? Through a robust review of the literature, the Task Force offers a statement on genetic sequencing, clinical management and screening of patients with FPF and their relatives. This proposal may serve as a basis for a prospective evaluation and future international recommendations., Competing Interests: Conflict of interest: R. Borie reports grants from Boehringer Ingelheim, consulting fees from Sanofi, lecture honoraria from Boehringer Ingelheim, Roche and Sanofi, and travel support from Boehringer Ingelheim, outside the submitted work. K. Antoniou reports consulting fees from Boehringer Ingelheim, Roche and GlaxoSmithKline, lecture honoraria from Boehringer Ingelheim, Roche, Chiesi, Menarini, GlaxoSmithKline and AstraZeneca, outside the submitted work. F. Bonella reports lecture honoraria from Boehringer Ingelheim, Fujirebio, Galapagos NV and Roche, travel support from Boehringer Ingelheim and Roche, participation on advisory boards with Boehringer Ingelheim, BMS, Fujirebio, Galapagos NV, GlaxoSmithKline, Roche and Takeda, outside the submitted work. B. Crestani reports grants from BMS, Boehringer Ingelheim and Roche, consulting fees from Apellis, lecture honoraria from Boehringer Ingelheim, BMS, Roche, Sanofi, Novartis, AstraZeneca and Chiesi, and is in receipt of equipment from Translate Bio, outside the submitted work. A. Froidure reports grants and consulting fees from Boehringer Ingelheim, lecture honoraria from Boehringer Ingelheim and GlaxoSmithKline, and travel support from Sanofi, outside the submitted work. L. Galvin reports travel support from the European Lung Foundation, European Respiratory Society and European Pulmonary Fibrosis Federation, leadership roles with the European Pulmonary Fibrosis Federation, Irish Lung Fibrosis Association, European Lung Foundation and European Reference Network On Rare Respiratory Diseases, outside the submitted work. M. Griese reports grants and travel support from Boehringer Ingelheim, outside the submitted work. M. Molina-Molina reports grants from Roche and Boehringer Ingelheim, consulting fees from Esteve-Teijin, Ferrer, Roche and Boehringer Ingelheim, and lecture honoraria from Chiesi, Roche and Boehringer Ingelheim, outside the submitted work. V. Poletti reports lecture honoraria from AMBU, Boehringer Ingelheim, ERBE and Roche, outside the submitted work. E. Renzoni reports grants from Boehringer Ingelheim, and lecture honoraria from Boehringer Ingelheim, Roche and Novartis, outside the submitted work. C.H.M. van Moorsel reports grants from the European Respiratory Society supporting the present manuscript, grants and lecture honoraria from Boehringer Ingelheim, and a leadership role as co-chair of the European Respiratory Society Task Force for genetics in pulmonary fibrosis, outside the submitted work. All other authors have nothing to disclose., (Copyright ©The authors 2023. For reproduction rights and permissions contact permissions@ersnet.org.)

Adult PAP patients experience similar #COVID19 rates to the general population, and high rates of hospitalisation and deaths, underscoring their vulnerability and the need for measures to prevent infection. The impact of iGM-CSF must be considered. https://bit.ly/3M0wKnZ., Competing Interests: Conflict of interest: S.A. Papiris reports grants, personal fees and nonfinancial support from La Roche Hoffman and Boehringer Ingelheim, and other support from Savara, outside the submitted work. Conflict of interest: F. Bonella reports grants and personal fees from Savara Pharma outside the submitted work. Conflict of interest: R. Borie has received consulting fees from Boehringer Ingelheim, Roche and Sanofi, outside the submitted work; payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events from Boehringer Ingelheim and Roche, outside the submitted work; support for attending meetings and/or travel received from Boehringer Ingelheim, Roche and Chiesi outside the submitted work; and participation on a data safety monitoring or advisory board for Savara, outside the submitted work. Conflict of interest: M. Molina-Molina reports personal fees and grants from Esteve-Teijin, Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: E. Jouneau reports personal fees and other support from Actelion, AIRB, AstraZeneca, Bellerophon Therapeutics, Biogen, BMS, Boehringer Ingelheim, Chiesi, FibroGen, Galecto Biotech, Genzyme, Gilead, GlaxoSmithKline, LVL, Mundipharma, Novartis, Pharm-Olam, Pfizer, Pliant Therapeutics, F. Hoffmann-La Roche, Ltd, Sanofi and Savara-Serendex, outside the submitted work. Conflict of interest: C. McCarthy is a scientific advisory board member of Savara Inc. unrelated to this work and has not received any payment from Savara Inc. for any work to date. Conflict of interest: E. Bendstrup reports grants and personal fees from Boehringer Ingelheim and Hofmann la Roche, and personal fees from Galapagos, outside the submitted work. Conflict of interest: M. Griese has received grants or contracts from Boehringer Ingelheim, outside the submitted work; has participated on a data safety or advisory board for Boehringer Ingelheim, outside the submitted work; and is the Head of chILD-EU, outside the submitted work. Conflict of interest: E.D. Manali reports personal fees and nonfinancial support from La Roche Hoffman; grants, personal fees and nonfinancial support from Boehringer Ingelheim; and other support from Savara, all outside the submitted work. Conflict of interest: The remaining authors have nothing to disclose., (Copyright ©The authors 2023.)

This article provides an overview of scientific highlights in the field of interstitial lung disease (ILD), presented at the virtual European Respiratory Society Congress 2021. A broad range of topics was discussed this year, ranging from translational and genetic aspects to novel innovations with the potential to improve the patient pathway. Early Career Members summarise a selection of interesting findings from different congress sessions, together with the leadership of Assembly 12 - Interstitial Lung Disease., Competing Interests: Conflict of interest: S.A. Guler reports payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim and Hoffmann-La Roche; and participation on a Data Safety Monitoring Board or Advisory Board for Boehringer Ingelheim (disclosures made outside the submitted work). Conflict of interest: S. Cuevas-Ocaña reports receiving support for attending meetings and/or travel from the ERS (disclosure made outside the submitted work). Conflict of interest: M. Nasser reports receiving consulting fees from Boehringer Ingelheim, AstraZeneca, Hoffman-La Roche and Chugai, outside the submitted work. Conflict of interest: W.A. Wuyts reports grants or contracts received form Roche, Boehringer Ingelheim, Galapagos, the NIH and Fund Scientific Research Flanders (disclosures made outside the submitted work). Conflict of interest: M.S. Wijsenbeek reports receiving grants or contracts from Hoffmann-La Roche and Boehringer Ingelheim; consulting fees from Roche, Boehringer Ingelheim, Galapagos, Bristol Myers Squibb, Galecto and Respivant; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim, Hoffmann-La Roche and Novartis; support for attending meetings and/or travel from Boehringer Ingelheim and Hoffmann-La Roche; and participation on a Data Safety Monitoring Board for Savara and Galapagos. Unpaid positions: Chair of the Idiopathic Interstitial Pneumonia Group of the ERS; member of the board of the Netherlands Respiratory Society; member of the scientific advisory board of the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation; Chair of the educational committee of the European Reference Network for Rare Lung Diseases; and advisory board of the Dutch lung fibrosis and sarcoidosis patient associations (disclosures made outside the submitted work). Conflict of interest: A. Froidure reports grants or contracts received from Roche and Boehringer Ingelheim; and consulting fees received from Boehringer Ingelheim and Roche (disclosures made outside the submitted work). Conflict of interest: E. Bargagli has nothing to disclose. Conflict of interest: E.A. Renzoni reports grants or contracts received from Boehringer Ingelheim; consulting fees received from Boehringer Ingelheim; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim, Roche and Chiesi; and support for attending meetings and/or travel received from Boehringer Ingelheim (disclosures made outside the submitted work). Conflict of interest: M. Veltkamp reports receiving consulting fees from Boehringer Ingelheim; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Chiesi Pharmaceutical; and participation on a Data Safety Monitoring Board or Advisory Board for Boehringer Ingelheim and the COLD study (disclosures made outside the submitted work). Conflict of interest: P. Spagnolo reports grants or contracts received from PPM Services, Roche and Boehringer Ingelheim; consulting fees received from PPM Services, Chiesi, Novartis and Pieris; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim, Roche and Chiesi; support for attending meetings and/or travel received from PPM Services and Boehringer Ingelheim; and participation on a Data Safety Monitoring Board or Advisory Board for Galapagos (disclosures made outside the submitted work). Conflict of interest: H. Nunes reports receiving consulting fees from Boehringer Ingelheim, Roche and Genentech; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim, Roche and Genentech; support for attending meetings and/or travel received from Boehringer Ingelheim, Roche and Genentech; participation on a Data Safety Monitoring Board or Advisory Board for Galapagos and Actelion Pharmaceuticals; and was an investigator of clinical trials for Sanofi, Gilead, Novartis, Galecto Biotech AB and BMS (disclosures made outside the submitted work). Conflict of interest: C. McCarthy reports receiving grants or contracts from Boehringer Ingelheim; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Roche Ltd and Aerogen; support for attending meetings and/or travel received from Boehringer Ingelheim and Roche; and participation on a Data Safety Monitoring Board or Advisory Board for Savara Inc. (disclosures made outside the submitted work). Conflict of interest: M. Molina-Molina reports grants or contracts received from Roche and Boehringer Ingelheim; consulting fees received from Esteve-Teijin, Ferrer and Chiesi; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim; and support for attending meetings and/or travel received from Boehringer Ingelheim (disclosures made outside the submitted work). Conflict of interest: F. Bonella reports receiving consulting fees from Boehringer Ingelheim and Savara Pharma; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Boehringer Ingelheim, Fujirebio, Galapagos NV and Roche; support for attending meetings and/or travel received from Boehringer Ingelheim and Roche; and participation on a Data Safety Monitoring Board or Advisory Board for Boehringer Ingelheim, Bristol Myers Squibb, Fujirebio, Galapagos NV, GlaxoSmithKline, Roche and Takeda (disclosures made outside the submitted work). Conflict of interest: V. Poletti has nothing to disclose. Conflict of interest: M. Kreuter reports receiving grants or contracts from Roche and Boehringer Ingelheim; consulting fees received from Roche, Boehringer Ingelheim and Galapagos; payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events received from Roche and Boehringer Ingelheim (disclosures made outside the submitted work). Conflict of interest: K.M. Antoniou reports receiving consulting fees from Boehringer Ingelheim and Roche; payment of honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events for Boehringer Ingelheim and Roche; and support for attending meetings and/or travel received from Boehringer Ingelheim and Roche; she is head of ERS Assembly 12 (disclosures made outside the submitted work). Conflict of interest: C.C. Moor reports receiving grants or contracts from Roche and Boehringer-Ingelheim; and speaker fees from Roche and Boehringer-Ingelheim. All grants and fees were paid to her institution (disclosures made outside the submitted work)., (Copyright ©The authors 2022.)

Communications between clinicians and patients with idiopathic pulmonary fibrosis (IPF) have the potential to be challenging. The variable course and poor prognosis of IPF complicate discussions around life expectancy but should not prevent clinicians from having meaningful conversations about patients' fears and needs, while acknowledging uncertainties. Patients want information about the course of their disease and management options, but the provision of information needs to be individualised to the needs and preferences of the patient. Communication from clinicians should be empathetic and take account of the patient's perceptions and concerns. Models, tools and protocols are available that can help clinicians to improve their interactions with patients. In this article, we consider the difficulties inherent in discussions with patients with IPF and their loved ones, and how clinicians might communicate with patients more effectively, from breaking the news about the diagnosis to providing support throughout the course of the disease., Competing Interests: Conflict of interest: M.S. Wijsenbeek reports grants, and non-financial and other support, with all grants and fees paid to her institution, from Boehringer Ingelheim and Hoffmann-La Roche; and other support, with all fees paid to her institution, from Galapagos NV, Novartis, Respivant and Savara. F. Bonella reports personal fees and non-financial support from Boehringer Ingelheim, Bristol-Myers Squibb, Fujirebio, Galapagos NV, GlaxoSmithKline and Roche. L. Orsatti is an employee of Boehringer Ingelheim. A-M. Russell reports grants and personal fees from Boehringer Ingelheim, and personal fees from Hoffmann-La Roche and the Irish Lung Fibrosis Association. C. Valenzuela reports personal fees from Boehringer Ingelheim, Bristol-Myers Squibb, Galapagos NV and Hoffmann-La Roche. W.A. Wuyts reports grants and other support, with all fees paid to his institution, from Boehringer Ingelheim, and grants, with all fees paid to his institution, from Roche. W.F. Baile reports fees for presentations from Boehringer Ingelheim., (Copyright ©The authors 2022.)

Background: The major reasons to treat sarcoidosis are to lower the morbidity and mortality risk or to improve quality of life (QoL). The indication for treatment varies depending on which manifestation is the cause of symptoms: lungs, heart, brain, skin or other manifestations. While glucocorticoids remain the first choice for initial treatment of symptomatic disease, prolonged use is associated with significant toxicity. Glucocorticoid-sparing alternatives are available. The presented treatment guidelines aim to provide guidance to physicians treating the very heterogenous sarcoidosis manifestations., Methods: A European Respiratory Society Task Force committee composed of clinicians, methodologists and patients with experience in sarcoidosis developed recommendations based on the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology. The committee developed eight PICO (Patients, Intervention, Comparison, Outcomes) questions and these were used to make specific evidence-based recommendations., Results: The Task Force committee delivered 12 recommendations for seven PICOs. These included treatment of pulmonary, cutaneous, cardiac and neurologic disease as well as fatigue. One PICO question regarding small-fibre neuropathy had insufficient evidence to support a recommendation. In addition to the recommendations, the committee provided information on how they use alternative treatments, when there was insufficient evidence to support a recommendation., Conclusions: There are many treatments available to treat sarcoidosis. Given the diverse nature of the disease, treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on QoL of the disease and treatment., Competing Interests: Conflict of interest: R.P. Baughman reports grants from Gilead, Genentech, Bayer, aTyr and Bellephron, grants and personal fees for consultancy from Novartis, personal fees for consultancy from Methial, grants and personal fees for consultancy and lectures from Mallinckrodt, grants and personal fees for lectures from Boehringer Ingelheim, outside the submitted work. Conflict of interest: D. Valeyre reports personal fees for advisory board work from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: P. Korsten reports grants and personal fees from GlaxoSmithKline, personal fees from AbbVie, Pfizer, Chugai, Novartis Pharma, Sanofi-Aventis, Lilly and Gilead, outside the submitted work. Conflict of interest: A.G. Mathioudakis reports grants from Boehringer Ingelheim, outside the submitted work. Conflict of interest: W.A. Wuyts has nothing to disclose. Conflict of interest: A. Wells reports personal fees for lectures and advisory board work from Boehringer Ingelheim and Roche, during the conduct of the study. Conflict of interest: P. Rottoli has nothing to disclose. Conflict of interest: H. Nunes has nothing to disclose. Conflict of interest: E.E. Lower reports grants from Gilead, Genentech, Bayer and aTYR, grants and personal fees for consultancy from Novartis, outside the submitted work. Conflict of interest: M.A. Judson received grant support for his institution from Mallickrodt. Conflict of interest: D. Israël-Biet has nothing to disclose. Conflict of interest: J.C. Grutters has nothing to disclose. Conflict of interest: M. Drent has nothing to disclose. Conflict of interest: D.A. Culver reports grants and personal fees from aTyr, Mallinckrodt, Boehringer Ingelheim and Foundation for Sarcoidosis Research, personal fees from Roche, outside the submitted work. Conflict of interest: F. Bonella reports personal fees and non-financial support from Boehringer Ingelheim, Roche, Galapagos, BMS and Savara Pharma, outside the submitted work. Conflict of interest: K. Antoniou has nothing to disclose. Conflict of interest: F. Martone has nothing to disclose. Conflict of interest: B. Quadder has nothing to disclose. Conflict of interest: G. Spitzer has nothing to disclose. Conflict of interest: B. Nagavci acts as an ERS methodologist. Conflict of interest: T. Tonia acts as an ERS methodologist. Conflict of interest: D. Rigau acts as an ERS methodologist. Conflict of interest: D.R. Oullette reports grants from PCORI (Patient-Centred Outcomes Research Institute, US Federal) and Sanofi, outside the submitted work; and has provided expert witness work for venous thromboembolic disease., (Copyright ©The authors 2021. For reproduction rights and permissions contact permissions@ersnet.org.)

Introduction: For the management of patients referred to respiratory triage during the early stages of the severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) pandemic, either chest radiography or computed tomography (CT) were used as first-line diagnostic tools. The aim of this study was to compare the impact on the triage, diagnosis and prognosis of patients with suspected COVID-19 when clinical decisions are derived from reconstructed chest radiography or from CT., Methods: We reconstructed chest radiographs from high-resolution CT (HRCT) scans. Five clinical observers independently reviewed clinical charts of 300 subjects with suspected COVID-19 pneumonia, integrated with either a reconstructed chest radiography or HRCT report in two consecutive blinded and randomised sessions: clinical decisions were recorded for each session. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and prognostic value were compared between reconstructed chest radiography and HRCT. The best radiological integration was also examined to develop an optimised respiratory triage algorithm., Results: Interobserver agreement was fair (Kendall's W =0.365, p<0.001) by the reconstructed chest radiography-based protocol and good (Kendall's W =0.654, p<0.001) by the CT-based protocol. NPV assisted by reconstructed chest radiography (31.4%) was lower than that of HRCT (77.9%). In case of indeterminate or typical radiological appearance for COVID-19 pneumonia, extent of disease on reconstructed chest radiography or HRCT were the only two imaging variables that were similarly linked to mortality by adjusted multivariable models CONCLUSIONS: The present findings suggest that clinical triage is safely assisted by chest radiography. An integrated algorithm using first-line chest radiography and contingent use of HRCT can help optimise management and prognostication of COVID-19., Competing Interests: Conflict of interests: N. Sverzellati has nothing to disclose. Conflict of interests: C.J. Ryerson has nothing to disclose. Conflict of interests: G. Milanese has nothing to disclose. Conflict of interests: E.A. Renzoni has nothing to disclose. Conflict of interests: A. Volpi has nothing to disclose. Conflict of interests: P. Spagnolo reports grants, personal fees and nonfinancial support from Roche, PPM Services and Boehringer Ingelheim, personal fees from Red X Pharma, Galapagos and Chiesi, outside the submitted work; and the author's spouse is an employee of Novartis. Conflict of interests: F. Bonella reports personal fees and nonfinancial support from Boehringer Ingelheim, Roche, Galapagos, Savara Pharma and Bristol Myers Squibb, outside the submitted work. Conflict of interests: I. Comelli has nothing to disclose. Conflict of interests: P. Affanni has nothing to disclose. Conflict of interests: L. Veronesi has nothing to disclose. Conflict of interests: C. Manna has nothing to disclose. Conflict of interests: A. Ciuni has nothing to disclose. Conflict of interests: C. Sartorio has nothing to disclose. Conflict of interests: G. Tringali has nothing to disclose. Conflict of interests: M. Silva has nothing to disclose. Conflict of interests: E. Michieletti has nothing to disclose. Conflict of interests: D. Colombi has nothing to disclose. Conflict of interests: A.U. Wells reports personal fees and other from Roche, Boehringer Ingelheim and Bayer, outside the submitted work., (Copyright ©The authors 2021.)

Patient-reported outcome measures (PROMs), tools to assess patient self-report of health status, are now increasingly used in research, care and policymaking. While there are two well-developed disease-specific PROMs for interstitial lung diseases (ILD) and idiopathic pulmonary fibrosis (IPF), many unmet and urgent needs remain. In December 2019, 64 international ILD experts convened in Erice, Italy to deliberate on many topics, including PROMs in ILD. This review summarises the history of PROMs in ILD, shortcomings of the existing tools, challenges of development, validation and implementation of their use in clinical trials, and the discussion held during the meeting. Development of disease-specific PROMs for ILD including IPF with robust methodology and validation in concordance with guidance from regulatory authorities have increased user confidence in PROMs. Minimal clinically important difference for bidirectional changes may need to be developed. Cross-cultural validation and linguistic adaptations are necessary in addition to robust psychometric properties for effective PROM use in multinational clinical trials. PROM burden of use should be reduced through appropriate use of digital technologies and computerised adaptive testing. Active patient engagement in all stages from development, testing, choosing and implementation of PROMs can help improve probability of success and further growth., Competing Interests: Conflict of interest: M. Kalluri reports grants and personal fees from Boehringer Ingelheim, and Roche, outside the submitted work. Conflict of interest: F. Luppi reports grants and lecture fees from Roche and lecture fees from Boehringer Ingelheim. Conflict of interest: A. Vancheri has nothing to disclose. Conflict of interest: C. Vancheri reports grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: E. Balestro reports personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: F. Varone reports unrestricted grants, consultancy and lecture fees from Roche and Boehringer Ingelheim. Conflict of interest: N. Mogulkoc has nothing to disclose. Conflict of interest: G. Cacopardo has nothing to disclose. Conflict of interest: E. Bargagli has nothing to disclose. Conflict of interest: E. Renzoni reports grants from Boeringher Ingelheim, and lecture fees from Boeringher Ingelheim and Roche, outside the submitted work. Conflict of interest: S. Torrisi reports personal fees from Hoffman La-Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: M. Calvello has nothing to disclose. Conflict of interest: A. Libra has nothing to disclose. Conflict of interest: M. Pavone has nothing to disclose. Conflict of interest: F. Bonella reports grants and personal fees from Boehringer Ingelheim and Roche, and consultancy fees from BMS, Galapagos, GSK and Savara, outside the submitted work. Conflict of interest: V. Cottin reports personal fees and non-financial support from Actelion and Roche/Promedior; grants, personal fees and non-financial support from Boehringer Ingelheim; and personal fees from Bayer/MSD, Novartis, Sanofi, Celgene/BMS, Galapagos, Galecto, Shionogi, AstraZeneca and Fibrogen, outside the submitted work. Conflict of interest: C. Valenzuela reports personal fees from Boehringer Ingelheim, F. Hoffmann-La Roche, Galapagos and BMS, outside the submitted work. Conflict of interest: M. Wijsenbeek reports grants and other funding from Boehringer Ingelheim and Hoffman-La Roche, and other funding from Respivant, Galapagos, Novartis and Savara, outside the submitted work. All fees and grants were paid to her institution. Conflict of interest: E. Bendstrup reports grants and personal fees from Boehringer Ingelheim and Hoffmann-La Roche, and personal fees from Galapagos and AstraZeneca, outside the submitted work., (Copyright ©The authors 2021.)

Background: Currently there is major lack of agreement on the diagnostic and therapeutic management of patients with idiopathic pulmonary fibrosis (IPF) and lung cancer. Our aim was to identify variations in diagnostic and management strategies across different institutions and provide rationale for a consensus statement on this issue., Methods: This was a joint-survey by European Respiratory Society (ERS) Assemblies 8, 11 and 12. The survey consisted of 25 questions., Results: Four hundred and ninety-four (n=494) physicians from 68 different countries and five continents responded to the survey. Ninety-four per cent of participants were pulmonologists, 1.8% thoracic surgeons and 1.9% oncologists; 97.7% were involved in multidisciplinary team approaches on diagnosis and management. Regular low-dose high-resolution computed tomography (HRCT) scan was used by 49.5% of the respondents to screen for lung cancer in IPF. Positron emission tomography (PET) scan and endobronchial ultrasound (EBUS) is performed by 60% and 88% to diagnose nodular lesions with mediastinal lymphadenopathy in patients with advanced and mild IPF, respectively. Eighty-three per cent of respondents continue anti-fibrotics following lung cancer diagnosis; safety precautions during surgical interventions including low tidal volume are applied by 67%. Stereotactic radiotherapy is used to treat patients with advanced IPF (diffusing capacity of the lung for carbon monoxide ( D LCO ) <35%) and otherwise operable nonsmall cell lung cancer (NSCLC) by 54% of respondents and doublet platinum regimens and immunotherapy for metastatic disease by 25% and 31.9%, respectively. Almost all participants (93%) replied that a consensus statement for the management of these patients is highly warranted., Conclusion: The diagnosis and management of IPF-lung cancer (LC) is heterogeneous with most respondents calling for a consensus statement., Competing Interests: Conflict of interest: A. Tzouvelekis has received travel grants and consultation fees from Boehringer Ingelheim and Hoffmann La Roche outside the submitted work. Conflict of interest: K. Antoniou has received travel grants and consultation fees from Boehringer Ingelheim and Hoffmann La Roche. Conflict of interest: M. Kreuter has nothing to disclose. Conflict of interest: M. Evison has nothing to disclose. Conflict of interest: T.G. Blum has nothing to disclose. Conflict of interest: V. Poletti has nothing to disclose. Conflict of interest: B. Grigoriu has nothing to disclose. Conflict of interest: C. Vancheri has received unrestricted grants, and speaker and advisory board fees from Boehringer Ingelheim and F. Hoffmann-La Roche Ltd. Conflict of interest: P. Spagnolo reports grants, personal fees and nonfinancial support from Roche, PPM Services and Boehringer-Ingelheim, and reports personal fees from Red X Pharma, Galapagos and Chiesi, outside of the submitted work. Conflict of interest: T. Karampitsakos has nothing to disclose. Conflict of interest: F. Bonella has received travel grants and consultation fees from Boehringer Ingelheim, Hoffmann La Roche, Galapalagos, Savara and BMS. Conflict of interest: A. Wells has received travel grants and consultation fees from Boehringer Ingelheim and Hoffmann La Roche. Conflict of interest: G. Raghu has nothing to disclose. Conflict of interest: M. Molina-Molina reports grants and payment for scientific advice for Roche, Boehringer Ingelheim, Esteve-Teijin, Chiesi, Pfizer, GSK and Galapagos. Conflict of interest: D. Culver has nothing to disclose. Conflict of interest: E. Bendstrup has nothing to disclose. Conflict of interest: N. Mogulkoc has nothing to disclose. Conflict of interest: S. Elia has nothing to disclose. Conflict of interest: J. Cadranel reports grants from BI, and personal fees for consultancy and experts boards from Roche and BI, outside the submitted work. Conflict of interest: D. Bouros reports grants, personal fees, nonfinancial support and other support from BI Hellas, and other support from Roche, outside the submitted work., (Copyright ©ERS 2021.)

This article discusses a selection of the scientific presentations in the field of interstitial lung diseases (ILDs) that took place at the 2019 European Respiratory Society International Congress in Madrid, Spain. There were sessions from all four groups within Assembly 12: group 12.01 "Idiopathic interstitial pneumonias", group 12.02 "ILDs/diffuse parenchymal lung diseases (DPLDs) of known origin", group 12.03 "Sarcoidosis and other granulomatous ILDs/DPLDs" and group 12.04 "Rare ILDs/DPLDs". The presented studies brought cutting-edge developments on several aspects of these conditions, including pathogenesis, diagnosis and treatment. As many of the ILDs are individually rare, the sharing of experiences and new data that occur during the Congress are very important for physicians interested in ILDs and ILD patients alike., Competing Interests: Conflict of interest: C. Dupin reports grants from AstraZeneca, GSK and Sanofi, personal fees and non-financial support from AstraZeneca, Chiesi, GSK, Roche and Sanofi, non-financial support from Boehringer, and personal fees from Novartis, all outside the submitted work. Conflict of interest: V. Fernandes has nothing to disclose. Conflict of interest: F. Hernandez-Gonzales has nothing to disclose. Conflict of interest: S.E. Torrisi has nothing to disclose. Conflict of interest: T.M. Alfaro has nothing to disclose. Conflict of interest: M. Kreuter reports grants and personal fees from Boehringer and Roche, and personal fees from Galapagos, outside the submitted work. Conflict of interest: M.S. Wijsenbeek reports grants from Boehringer Ingelheim and Hoffman la Roche, and other support from Boehringer Ingelheim, Hoffman la Roche, Galapagos and Respivant, all paid to her institution and all outside the submitted work. Conflict of interest: E.A. Renzoni reports lecture and advisory board fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: E. Bargagli has nothing to disclose. Conflict of interest: H. Nunes reports consultancy and research support fees from Roche/Genentech and Boehringer Ingelheim, personal fees for serving as an expert for a clinical end-point committee from Galapagos, and other support for serving as an investigator on clinical trials from Sanofi, Gilead, Novartis and Galecto Biotech AB, and for service as a board expert on a clinical trial from Actelion Pharmaceuticals, during the conduct of the study. Conflict of interest: P. Spagnolo reports grants, personal fees and non-financial support from Roche, PPM Services and Boehringer Ingelheim, and personal fees from Red X Pharma, Galapagos and Chiesi, all outside the submitted work; additionally, his wife is an employee of Novartis. Conflict of interest: F. Bonella reports grants, personal fees and non-financial support from Boehringer Ingelheim, Roche and Galapagos, and grants and personal fees from Savara, outside the submitted work. Conflict of interest: M. Molina-Molina reports grants and personal fees from Roche, Boehringer Ingelheim and Esteve-Teijin, and personal fees from Chiesi and Pfizer, outside the submitted work. Conflict of interest: K. Antoniou has nothing to disclose. Conflict of interest: V. Poletti has nothing to disclose., (Copyright ©ERS 2020.)

Introduction: Pulmonary fibrosis (PF) and its most common form, idiopathic pulmonary fibrosis (IPF), are chronic, progressive diseases resulting in increasing loss of lung function and impaired quality of life and survival. The aim of this joint expert and patient statement was to highlight the most pressing common unmet needs of patients with PF/IPF, putting forward recommendations to improve the quality of life and health outcomes throughout the patient journey., Methods: Two online surveys for patients and healthcare professionals (HCPs) were conducted by the European Idiopathic Pulmonary Fibrosis and Related Disorders Federation (EU-IPFF) in 14 European countries., Results: The surveys were answered by 286 patients and 69 HCPs, including physicians and nurses. Delays in diagnosis and timely access to interstitial lung disease specialists and pharmacological treatment have been identified as important gaps in care. Additionally, patients and HCPs reported that a greater focus on symptom-centred management, adequate information, trial information and increasing awareness of PF/IPF is required., Conclusions: The surveys offer important insights into the current unmet needs of PF/IPF patients. Interventions at different points of the care pathway are needed to improve patient experience., Competing Interests: Conflict of interest: C.C. Moor has nothing to disclose. Conflict of interest: M.S. Wijsenbeek reports grants and other fees paid to her institution by Hoffmann-La Roche and Boehringer Ingelheim, and other fees paid to her institution by Galapagos, outside the submitted work. Conflict of interest: E. Balestro has nothing to disclose. Conflict of interest: D. Biondini has nothing to disclose. Conflict of interest: B. Bondue reports grants and personal fees from Hoffmann-La Roche and Boehringer Ingelheim outside the submitted work. Conflict of interest: V. Cottin reports consultancy fees, lecture fees and travel expenses to medical meetings from Actelion and Boehringer Ingelheim, consultancy fees from Bayer/MSD, personal fees for membership of an adjudication committee from Gilead, consultancy fees and lectures fees from Novartis, consultancy fees, lecture fees, travel to medical meetings and a grant to his institution from Roche, speaker fees from Sanofi, personal fees for acting as the chair of a data and safety monitoring board (DSMB) from Promedior, personal fees for membership of DSMBs from Celgene and Galecto, and personal fees for consultancy and membership of a DSMB from Galapagos, outside the submitted work. Conflict of interest: R. Flewett has nothing to disclose. Conflict of interest: L. Galvin has nothing to disclose. Conflict of interest: S. Jones takes part in the annual Patient Advocacy Group meeting organised by Boehringer Ingelheim, who pay a nominal amount to Action for Pulmonary Fibrosis (APF). He organises some meetings with patients for which small honoraria are paid to APF by Boehringer Ingelheim. He has also arranged meetings for Galapagos, Roche, GSK and Vicore with patients, for which an honorarium was sometimes paid to APF. Conflict of interest: M. Molina-Molina reports grants and personal fees from Roche, Boehringer and Esteve-Teijin, grants from GSK, and personal fees from Pfizer, outside the submitted work. Conflict of interest: L. Planas-Cerezales has nothing to disclose. Conflict of interest: A. Prasse reports personal fees and nonfinancial support from Boehringer Ingelheim, Roche, AstraZeneca, Novartis, SanofiAventis and NittoDenko, and personal fees from AbbVie and Pliant, outside the submitted work. Conflict of interest: H. Prosch reports grants and personal fees from Boehringer Ingelheim, and personal fees from Roche, during the conduct of the study. Conflict of interest: A-M. Russell reports expenses and hospitality to attend scientific advisory board meetings from EU-IPFF, personal fees from ILFA, grants and nonfinancial support from Hoffmann-La Roche and nonfinancial support from Boehringer Ingelheim during the conduct of the study; and grants from Pulmonary Fibrosis Trust UK and nonfinancial support from the ILD Interdisciplinary Network outside the submitted work. Conflict of interest: M. Viegas has nothing to disclose. Conflict of interest: G. Wanke has nothing to disclose. Conflict of interest: W. Wuyts reports grants pay to his university by Roche and Boehringer Ingelheim outside the submitted work. Conflict of interest: M. Kreuter reports personal fees from Galapagos, and grants and personal fees from Roche and Boehringer Ingelheim, outside the submitted work. Conflict of interest: F. Bonella reports grants, personal fees and nonfinancial support from Boehringer Ingelheim, Roche and Savara Pharma, outside the submitted work., (Copyright ©ERS 2019.)

Nintedanib is a tyrosine kinase inhibitor used to treat idiopathic pulmonary fibrosis (IPF). We investigated the cardiovascular safety of nintedanib using pooled data from the TOMORROW and INPULSIS trials.Cardiovascular events were assessed post hoc in patients with a history of atherosclerotic cardiovascular disease (CVD) and/or one or more cardiovascular risk factors at baseline ("higher cardiovascular risk") and patients with no history of atherosclerotic CVD and no cardiovascular risk factors at baseline ("lower cardiovascular risk").Incidence rates were calculated for 1231 patients (n=723 nintedanib and n=508 placebo), of whom 89.9% had higher cardiovascular risk. Incidence rates of major adverse cardiovascular events were similar in the nintedanib and placebo groups in patients with higher cardiovascular risk (3.88 (95% CI 2.58-5.84) and 3.49 (95% CI 2.10-5.79) per 100 patient-years, respectively) and lower cardiovascular risk (4.78 (95% CI 1.54-14.82) and 5.37 (95% CI 1.73-16.65) per 100 patient-years, respectively). Incidence rates of myocardial infarction in the nintedanib and placebo groups, respectively, were 3.03 (95% CI 1.91-4.81) and 1.16 (95% CI 0.48-2.79) per 100 patient-years in patients with higher cardiovascular risk and 1.59 (95% CI 0.22-11.29) and 1.78 (95% CI 0.25-12.64) per 100 patient-years in patients with lower cardiovascular risk. Incidence rates of other ischaemic heart disease in the nintedanib and placebo groups, respectively, were 1.85 (95% CI 1.02-3.34) and 3.28 (95% CI 1.94-5.54) per 100 patient-years in patients with higher cardiovascular risk and 0 and 1.80 (95% CI 0.25-12.78) per 100 patient-years in patients with lower cardiovascular risk.These data help to establish the cardiovascular safety profile of nintedanib in IPF., Competing Interests: Conflict of interest: I. Noth reports personal fees for consultancy, lecturing, advisory board work and travel support from Boehringer Ingelheim and Roche/Genentech, personal fees for advisory board work from Sanofi and Global Blood Therapeutics, and personal fees for consultancy from Veracyte, outside the submitted work. Conflict of interest: M. Wijsenbeek reports grants and other from Boehringer Ingelheim and Roche, and other from Galapagos, outside the submitted work. Conflict of interest: M. Kolb reports grants from Canadian Pulmonary Fibrosis Foundation and Canadian Institute for Health Research, has acted as site principal investigator in industry-sponsored clinical trials for Roche, Sanofi and Boehringer Ingelheim, grants from and advisory board work for Pulmonary Fibrosis Foundation, personal fees for advisory board work from Boehringer Ingelheim, GlaxoSmithKline, AstraZeneca, Vertex, Genoa, Gilead, Prometic and Alkermes, and grants and personal fees for advisory board work from Roche Canada and Janssen, outside the submitted work. Conflict of interest: F. Bonella reports personal fees for consultancy, lecturing and travel support from Boehringer Ingelheim and Roche, outside the submitted work. Conflict of interest: L. Moros is an employee of Boehringer Ingelheim. Conflict of interest: D. Wachtlin is an employee of Boehringer Ingelheim. Conflict of interest: T.J. Corte reports grants and personal fees for travel, lecturing and advisory board compensation from Boehringer Ingelheim, grants and personal fees for travel, lecturing, steering committee work and advisory board compensation from Roche, grants from Gilead, Bayer, Intermune and BMS, and personal fees for advisory board compensation from AstraZeneca, during the conduct of the study., (Copyright ©ERS 2019.)

This article reviews a selection of the scientific presentations on interstitial lung disease (ILD)/diffuse parenchymal lung disease (DPLD) that were made at the 2018 European Respiratory Society (ERS) International Congress in Paris. A number of advances in the epidemiology, pathogenesis, diagnosis and treatment of these disorders were presented and discussed by clinicians and researchers. The research topics span over all four groups of ERS Assembly 12: Interstitial Lung Diseases (Group 12.01: Idiopathic interstitial pneumonias; Group 12.02: ILD/DPLD of known origin; Group 12.03: Sarcoidosis and other granulomatous ILD/DPLD; Group 12.04: Rare ILD/DPLD)., Competing Interests: Conflict of interest: T.M. Alfaro reports receiving travel support from Boehringer Ingelheim, Novartis, Astra, Menarini, Mundipharma, Zambon and Roche, and grants from Bayer, outside the submitted work. Conflict of interest: C.C. Moor has nothing to disclose. Conflict of interest: V. Alfieri has nothing to disclose. Conflict of interest: F. Jeny has nothing to disclose. Conflict of interest: M. Kreuter has nothing to disclose. Conflict of interest: M.S. Wijsenbeek reports grants and other support from Boehringer Ingelheim and Hoffman la Roche, and other support from Galapagos, outside the submitted work. Conflict of interest: E.A. Renzoni reports lecture and advisory board fees from Roche and Boehringer Ingelheim, and lecture fees from Mundipharma, outside the submitted work. Conflict of interest: E. Bargagli has nothing to disclose. Conflict of interest: H. Nunes reports consultancy and research support fees from Roche/Genentech and Boehringer Ingelheim, and grants and personal fees as a clinical trial investigator from Sanofi and Gilead. Conflict of interest: P. Spagnolo reports personal fees and nonfinancial support from Roche, Boehringer Ingelheim and Zambon, personal fees from PPM Services, Galapagos and Santhera Pharmaceuticals, outside the submitted work. Conflict of interest: F. Bonella reports personal fees and nonfinancial support from Roche Pharma, Boehringer Ingelheim and Fujrebio, outside the submitted work. Conflict of interest: M. Molina-Molina reports grants from Roche, Boehringer Ingelheim and grants from Esteve-Teijin Healthcare, and personal fees for specialised medical training from Chiesi, outside the submitted work. Conflict of interest: K. Antoniou has nothing to disclose. Conflict of interest: V. Poletti has nothing to disclose.

Sarcoidosis is a highly variable, systemic granulomatous disease of hitherto unknown aetiology. The GenPhenReSa (Genotype-Phenotype Relationship in Sarcoidosis) project represents a European multicentre study to investigate the influence of genotype on disease phenotypes in sarcoidosis.The baseline phenotype module of GenPhenReSa comprised 2163 Caucasian patients with sarcoidosis who were phenotyped at 31 study centres according to a standardised protocol.From this module, we found that patients with acute onset were mainly female, young and of Scadding type I or II. Female patients showed a significantly higher frequency of eye and skin involvement, and complained more of fatigue. Based on multidimensional correspondence analysis and subsequent cluster analysis, patients could be clearly stratified into five distinct, yet undescribed, subgroups according to predominant organ involvement: 1) abdominal organ involvement, 2) ocular-cardiac-cutaneous-central nervous system disease involvement, 3) musculoskeletal-cutaneous involvement, 4) pulmonary and intrathoracic lymph node involvement, and 5) extrapulmonary involvement.These five new clinical phenotypes will be useful to recruit homogenous cohorts in future biomedical studies., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at erj.ersjournals.com, (Copyright ©ERS 2018.)

This article contains highlights and a selection of the scientific advances from the European Respiratory Society's Clinical Assembly (Assembly 1 and its six respective groups) that were presented at the 2017 European Respiratory Society International Congress in Milan, Italy. The most relevant topics from each of the groups will be discussed, covering a wide range of areas including clinical problems, rehabilitation and chronic care, thoracic imaging, interventional pulmonology, diffuse and parenchymal lung diseases, and general practice and primary care. In this comprehensive review, the newest research and actual data as well as award-winning abstracts and highlight sessions will be discussed., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at openres.ersjournals.com

Granulomatous lung diseases are a heterogeneous group of disorders that have a wide spectrum of pathologies with variable clinical manifestations and outcomes. Precise clinical evaluation, laboratory testing, pulmonary function testing, radiological imaging including high-resolution computed tomography and often histopathological assessment contribute to make a confident diagnosis of granulomatous lung diseases. Differential diagnosis is challenging, and includes both infectious (mycobacteria and fungi) and noninfectious lung diseases (sarcoidosis, necrotising sarcoid granulomatosis, hypersensitivity pneumonitis, hot tub lung, berylliosis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, rheumatoid nodules, talc granulomatosis, Langerhans cell histiocytosis and bronchocentric granulomatosis). Bronchoalveolar lavage, endobronchial ultrasound-guided transbronchial needle aspiration, transbronchial cryobiopsy, positron emission tomography and genetic evaluation are potential candidates to improve the diagnostic accuracy for granulomatous lung diseases. As granuloma alone is a nonspecific histopathological finding, the multidisciplinary approach is important for a confident diagnosis., Competing Interests: Conflict of interest: Disclosures can be found alongside this article at err.ersjournals.com, (Copyright ©ERS 2017.)

YKL-40, a chitinase-like protein mainly secreted by macrophages, neutrophils and epithelial cells, is increased in patients with idiopathic interstitial pneumonia and sarcoidosis. We aimed to investigate the role of YKL-40 as a biomarker in hypersensitivity pneumonitis (HP).72 HP patients, 100 interstitial lung disease (ILD) controls and 60 healthy controls were studied. YKL-40 was measured by ELISA in serum and bronchoalveolar lavage fluid (BALF) at baseline and follow-up. The relationship between YKL-40 levels, clinical variables and disease outcome was evaluated.Baseline serum YKL-40 levels were significantly higher in HP patients than in healthy controls (p<0.001), but lower than in patients with other ILDs. Baseline BALF YKL-40 levels in HP patients were the highest among ILD patients. In HP patients, serum YKL-40 correlated with the diffusing capacity of the lung for carbon monoxide at baseline (p<0.01) and over time (p<0.001). HP patients whose disease progressed or who died had higher baseline YKL-40 levels than those who remained stable and survived (p<0.001). At a cut-off of 119 ng·mL -1 , the baseline serum YKL-40 level predicted disease progression (hazard ratio 6.567; p<0.001), and at a cut-off of 150 ng·mL -1 was associated with mortality (hazard ratio 9.989; p<0.001).Serum YKL-40 may be a useful prognostic biomarker in HP patients., (Copyright ©ERS 2017.)

Patient advocacy groups play an important role in supporting patients with chronic diseases and promoting better care. The aim of this patient-physician initiative was to gather perceptions from European idiopathic pulmonary fibrosis (IPF) patient advocacy groups regarding inequalities and unmet needs in IPF care, in order to develop a Patient Charter to advocate for better care.In total, 11 European patient advocacy groups were interviewed regarding the care of patients with IPF in their countries. Interview feedback was presented to a Working Group including patient advocacy group representatives and IPF specialists; key areas of agreement were developed into the European IPF Patient Charter.The interviews identified five key themes that fed into the final Charter: the need for improved diagnosis, treatment access, holistic care, disease awareness and palliative care. The final Charter was endorsed by patient advocacy groups and presented to 26 Members of the European Parliament in September 2014. It has received >8900 signatures to date.This patient-physician initiative highlights the inequalities and unmet needs in IPF care across Europe, and demonstrates how this insight can inform the development of a Patient Charter, designed as a call to action for healthcare policymakers to drive improvement in European IPF care., (Copyright ©ERS 2016.)

Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug toxicity, asbestosis, familial IPF and Hermansky-Pudlak syndrome. Differentiating IPF ("idiopathic UIP") from conditions that mimic IPF ("secondary UIP") has substantial therapeutic and prognostic implications. A number of radiological and histological clues may help distinguish IPF from other conditions with a UIP pattern of fibrosis, but their appreciation requires extensive expertise in interstitial lung disease as well as an integrated multidisciplinary approach involving pulmonologists, radiologists and pathologists. In addition, multidisciplinary discussions may decrease the time to initial IPF diagnosis and, thus, enable more timely management. This concept was strongly emphasised by the 2011 ATS/ERS/JRS/ALAT guidelines. This article highlights, with the aid of a clinical case, the difficulties in making a diagnosis of IPF in clinical practice. Yet, an accurate diagnosis is critical, particularly given the availability of drugs that may reduce the pace of functional decline and disease progression in IPF., (©ERS 2014.)

Interstitial lung diseases are a group of diffuse parenchymal lung disorders associated with substantial morbidity and mortality. Knowledge achieved in recent years has resulted in the publication of the new classification of idiopathic interstitial pneumonias, according to which there are three groups: major, rare and unclassified. The novelty of the new classification comes from the fact that difficult to classify entities can be treated according to the disease behaviour classification. Idiopathic pulmonary fibrosis is the most lethal amongst the interstitial lung diseases and presents high heterogeneity in clinical behaviour. A number of biomarkers have been proposed in order to predict the course of the disease and group patients with the same characteristics in clinical trials. Early diagnosis and disease stratification is also important in the field of other interstitial lung diseases.

Whole lung lavage (WLL) is the standard treatment for pulmonary alveolar proteinosis (PAP). This study aimed to provide data about the kinetics of the protein wash-out, to identify factors influencing the protein concentration in the recovered fluid and to assess the efficacy of a modified lavage technique. Samples of 180 WLLs from 42 adult PAP patients were collected. 110 WLLs were performed according to the classical technique. In 70 WLLs, repeated manual ventilation was applied during the procedure. Spectrophotometry was used to measure the protein concentration in the recovered fluid. The initial protein concentration in the recovered fluid was 460 mg · dL(-1), the final concentration was 26 mg · dL(-1) and the total amount of removed proteins during a lavage was 17.5 g. A history of dust exposure was associated with a higher residual protein concentration in the recovered fluid (p=0.000013). The amount of removed proteins correlated inversely with the diffusing capacity of the lung for carbon monoxide (p=0.001) and oxygen tension (p=0.004). The modified technique removed a greater amount of proteins than the classical technique and prolonged the time to relapse (p=0.011). Exposure to dust seems to influence the kinetics of the protein wash-out. Applying manual ventilation during the procedure can enhance the efficacy of WLL.