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Competing Interests: Katharina Buschulte erhielt Vortragshonorare von Boehringer Ingelheim. Klaas Franzen gibt AOP, Chiesi und Inogen als Interessenkonflikte an. Gabriela Leuschner gibt Reise- und Übernachtungskosten, Vortrags- und Beratertätigkeit von Boehringer Ingelheim an. Nina Sicker erhielt Reise- und Übernachtungskosten von Astra Zeneca, Chiesi und Boehringer Ingelheim. Finn Wilkens gibt Boehringer Ingelheim, CSL Behring, Berlin Chemie und DZL als Interessenkonflikte an. Christoph Fisser gibt Reise- und Übernachtungskosten, Vortrags- und Beratertätigkeit von AstraZeneca, Berlin-Chemie, CSL Behring, Chiesi und Novartis an sowie Forschungsunterstützung der deutschen Stiftung für Herzforschung außerhalb der eingereichten Arbeit. Erik Büscher, Armin Frille, Espen Groth, Julia Hoffmann, Andreas Knaack, Johannes Krönig, Christoph Lederer und Eva Pappe geben keine Interessenkonflikte an.

Background: Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with different disease trajectories. Progression (PF-ILD) occurs in up to 50% of patients and is associated with increased mortality., Methods: The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for disease trajectories in different ILD. The course of disease was classified as significant (absolute forced vital capacity FVC decline > 10%) or moderate progression (FVC decline 5-10%), stable disease (FVC decline or increase < 5%) or improvement (FVC increase ≥ 5%) during time in registry. A second definition for PF-ILD included absolute decline in FVC % predicted ≥ 10% within 24 months or ≥ 1 respiratory-related hospitalisation. Risk factors for progression were determined by Cox proportional-hazard models and by logistic regression with forward selection. Kaplan-Meier curves were utilised to estimate survival time and time to progression., Results: Within the EXCITING-ILD registry 28.5% of the patients died (n = 171), mainly due to ILD (n = 71, 41.5%). Median survival time from date of diagnosis on was 15.5 years (range 0.1 to 34.4 years). From 601 included patients, progression was detected in 50.6% of the patients (n = 304) with shortest median time to progression in idiopathic NSIP (iNSIP; median 14.6 months) and idiopathic pulmonary fibrosis (IPF; median 18.9 months). Reasons for the determination as PF-ILD were mainly deterioration in lung function (PFT; 57.8%) and respiratory hospitalisations (40.6%). In multivariate analyses reduced baseline FVC together with age were significant predictors for progression (OR = 1.00, p < 0.001). Higher GAP indices were a significant risk factor for a shorter survival time (GAP stage III vs. I HR = 9.06, p < 0.001). A significant shorter survival time was found in IPF compared to sarcoidosis (HR = 0.04, p < 0.001), CTD-ILD (HR = 0.33, p < 0.001), and HP (HR = 0.30, p < 0.001). Patients with at least one reported ILD exacerbation as a reason for hospitalisation had a median survival time of 7.3 years (range 0.1 to 34.4 years) compared to 19.6 years (range 0.3 to 19.6 years) in patients without exacerbations (HR = 0.39, p < 0.001)., Conclusion: Disease progression is common in all ILD and associated with increased mortality. Most important risk factors for progression are impaired baseline forced vital capacity and higher age, as well as acute exacerbations and respiratory hospitalisations for mortality. Early detection of progression remains challenging, further clinical criteria in addition to PFT might be helpful., (© 2024. The Author(s).)

The present recommendations on the therapy of sarcoidosis of the German Respiratory Society (DGP) was written in 2023 as a German-language supplement and update of the international guidelines of the European Respiratory Society (ERS) from 2021. It contains 5 PICO questions (Patients, Intervention, Comparison, Outcomes) agreed in the consensus process, which are explained in the background text of the four articles: Confirmation of diagnosis and monitoring of the disease under therapy, general therapy recommendations, therapy of cutaneous sarcoidosis, therapy of cardiac sarcoidosis., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Georg Thieme Verlag KG Rüdigerstraße 14, 70469 Stuttgart, Germany.)

Background: Interstitial lung diseases (ILD) comprise a heterogeneous group of mainly chronic lung diseases with more than 200 entities and relevant differences in disease course and prognosis. Little data is available on hospitalisation patterns in ILD., Methods: The EXCITING-ILD (Exploring Clinical and Epidemiological Characteristics of Interstitial Lung Diseases) registry was analysed for hospitalisations. Reasons for hospitalisation were classified as all cause, ILD-related and respiratory hospitalisations, and patients were analysed for frequency of hospitalisations, time to first non-elective hospitalisation, mortality and progression-free survival. Additionally, the risk for hospitalisation according to GAP index and ILD subtype was calculated by Cox proportional-hazard models as well as influencing factors on prediction of hospitalisation by logistic regression with forward selection., Results: In total, 601 patients were included. 1210 hospitalisations were recorded during the 6 months prior to registry inclusion until the last study visit. 800 (66.1%) were ILD-related, 59.3% of admissions were registered in the first year after inclusion. Mortality was associated with all cause, ILD-related and respiratory-related hospitalisation. Risk factors for hospitalisation were advanced disease (GAP Index stages II and III) and CTD (connective tissue disease)-ILDs. All cause hospitalisations were associated with pulmonary hypertension (OR 2.53, p = 0.005). ILD-related hospitalisations were associated with unclassifiable ILD and concomitant emphysema (OR = 2.133, p = 0.001) as well as with other granulomatous ILDs and a positive smoking status (OR = 3.082, p = 0.005)., Conclusion: Our results represent a crucial contribution in understanding predisposing factors for hospitalisation in ILD and its major impact on mortality. Further studies to characterize the most vulnerable patient group as well as approaches to prevent hospitalisations are warranted., (© 2023. The Author(s).)

Having sarcoidosis often has a major impact on quality of life of patients and their families. Improving quality of life is prioritized as most important treatment aim by many patients with sarcoidosis, but current evidence and treatment options are limited. In this narrative review, we describe the impact of sarcoidosis on various aspects of daily life, evaluate determinants of health-related quality of life (HRQoL), and provide an overview of the different patient-reported outcome measures to assess HRQoL in sarcoidosis. Moreover, we review the current evidence for pharmacological and non-pharmacological interventions to improve quality of life for people with sarcoidosis., Competing Interests: Declaration of competing interest VK and KB have nothing to disclose. CCM has no conflicts of interest related to the submitted work. Outside the submitted work she received grants and other from Boehringer Ingelheim, Hoffman la Roche, Astra Zeneca and Daiichi Sankyo. ONO has no conflicts of interest related to the submitted work. Outside the submitted work she received consulting fees from CSL-Behring, honoraria from Milliken Institute, fees and other from aTYR, Novartis, Kinevant, Xentria, and is a member of the advisory board of the Foundation for Sarcoidosis Research. MSW has no conflicts of interest related to the submitted work. Outside the submitted work she received grants and other from Bristol-Myers Squibb, Boehringer Ingelheim, Galapagos, Galecto, Hoffman la Roche, Horizon therapeutics, Kinevant Sciences, Molecure, Nerre Therapeutics, Novartis, PureTech Health, Thyron, Trevi, Vicore, CSL Behring, Savara. She is a member of the Advisory Board of the Dutch Sarcoidosis Patient Association., (Copyright © 2023 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: Many patients use the internet as a source of health information. Sarcoidosis is a complex disease, and internet resources have not yet been analyzed for reliability and content on sarcoidosis., Aims: Our study aimed to investigate the content and the quality of information on sarcoidosis provided by internet resources., Methods: Google, Yahoo, and Bing were searched for the term "sarcoidosis," and the first 200 hits were saved in each case. Those websites that met the inclusion criteria (English language, no registration fees, and relevant to sarcoidosis) were then analyzed by two independent investigators for readability, quality (HON, JAMA, and DISCERN), and content (25 predefined key facts) of the provided information., Results: The websites were most commonly scientific or governmental ( n = 57, 46%), and the median time since the last update was 24 months. Quality was rated with a median JAMA score of 2 (1; 4) and a median overall DISCERN score of 2.4 (1.1; 4.1), both scores represent partially sufficient information. In total, 15% of websites had a HON certificate. Website content measured by the median key fact score was 19 (ranging from 2.5 to 25) with the lowest scores for acute vs. chronic course of the disease, screening for extrapulmonary disease, and diffuse body pain. Poor results were achieved in industry websites and blogs ( p = 0.047) with significant differences regarding definition ( p = 0.004) and evaluation ( p = 0.021)., Discussion: Sarcoidosis-related content of internet resources is partially sufficient; however, several important aspects are frequently not addressed, and the quality of information is moderate. Future directions should focus on providing reliable and comprehensive information on sarcoidosis; physicians from different disciplines and patients including self-support groups should collaborate on achieving this., Competing Interests: KB received payment for lectures from Boehringer Ingelheim and a grant from Sarkoidose-Netzwerk e.V. JF has received grant funding from the University of Toronto and the Canadian Pulmonary Fibrosis Foundation and has received honoraria from Boehringer Ingelheim and AstraZeneca. CR reports grants from Boehringer Ingelheim and Hoffman la Roche; consulting fees from Boehringer Ingelheim, Hoffman la Roche, Astra Zeneca, Veracyte, Ensho, and Pliant Therapeutics; payment for lectures from Boehringer Ingelheim, Hoffman la Roche, and Cipla Ltd.; and support for attending meetings from Cipla Ltd., and Boehringer Ingelheim (unrelated to this submission). FH reports payment for lectures from Astra Zeneca, GSK, and Chiesi and is a part of the Data Safety Monitoring Board from Apogenix. MK reports grants, consulting fees, and payment for lectures from Boehringer Ingelheim and Roche. KK was employed by Limbach Gruppe SE. The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Buschulte, Höger, Ganter, Wijsenbeek, Kahn, Kriegsmann, Wilkens, Fisher, Ryerson, Herth and Kreuter.)

Introduction: Conservative management is usually preferred for iatrogenic tracheal injuries. Venovenous extracorporeal membrane oxygenation (V-V ECMO) is mostly used in acute refractory hypoxemia, airway lesions are an alternative indication., Case Report: A 51-year-old female was transferred with a large tracheal tear after plastic tracheotomy. Due to a critical ventilation situation with hypercapnia, conservative management was set and V-V ECMO was installed. With optimized tube positioning, minimal ventilation and gas transfer via V-V ECMO, a complete healing of the injury could be achieved., Discussion: Fast diagnosis of tracheal injuries is essential; transfer to a specialized centre should be considered. In our case, organ support via ECMO was necessary due to a difficult ventilation situation with persisting hypercapnia. Thus, reduction in ventilation pressures with reduction of possible leakage and healing of the tracheal tear could be achieved., Conclusion: Management of tracheal tears is complex; in severe cases special therapy concepts such as the use of V-V ECMO may become necessary., 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.

Interstitial lung diseases (ILD) comprise a heterogeneous group of chronic lung disorders of different etiologies that can not only affect the interstitium but also the alveolar space and the bronchial system. According to the "Global Burden of Disease Study" there has been an increase in incidence over the last decades and it is expected that the number of ILD-associated deaths will double over the next 20 years. ILD are grouped into those of unknown cause, e.g. idiopathic pulmonary fibrosis (IPF), and ILD of known cause, which include drug-induced and connective tissue disease-associated ILD as well as granulomatous ILD such as sarcoidosis and hypersensitivity pneumonitis. In addition, some ILD present a progressive fibrosing phenotype, which influences therapeutic decisions. Predominantly inflammatory entities are treated with immunosuppressives, whereas predominantly fibrosing ILD are treated with antifibrotic drugs; in some cases, a combination of both is necessary. The spectrum of differential diagnoses in ILD is broad, but definite diagnosis is essential for treatment selection; therefore, the multidisciplinary ILD board plays a pivotal role., (© 2023. The Author(s), under exclusive licence to Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)

The world of rare interstitial lung diseases (ILDs) is diverse and complex. Diagnosis and therapy usually pose challenges. This review describes a selection of rare and ultrarare ILDs including pulmonary alveolar proteinosis, pulmonary alveolar microlithiasis and pleuroparenchymal fibroelastosis. In addition, monogenic ILDs or ILDs in congenital syndromes and various multiple cystic lung diseases will be discussed. All these conditions are part of the scope of the European Reference Network on rare respiratory diseases (ERN-LUNG). Epidemiology, pathogenesis, diagnostics and treatment of each disease are presented., Competing Interests: Conflict of interest: K. Buschulte has received payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from BI. Conflict of interest: V. Cottin has received an unrestricted grant to his institution from Boehringer Ingelheim; consulting fees from Boehringer Ingelheim, Roche, Shionogi, RedX, Pure Tech, Celgene/BMS, AstraZeneca, CSL Behring, Sanofi, United Therapeutics/Ferrer and Pliant; fees for lectures from Boehringer Ingelheim and Roche; support for attending meetings from Boehringer Ingelheim and Roche; reports participation on a Data Safety Monitoring board for Galapagos and Galecto; and participated in an Adjudication committee for Fibrogen. Conflict of interest: M. Wijsenbeek has received payment for a DSMB from savarapharma. Conflict of interest: M. Kreuter has received grants or contracts, consulting fees and payment or honoraria for lectures, presentations, speakers’ bureaus, manuscript writing or educational events from BI and Roche; and has leadership or fiduciary roles at ERS, EU IPFF and DGP. Conflict of interest: R. Diesler has received support for attending meetings and/or travel from Laidet Medical/Asdia and Vitalaire; and has other financial or non-financial interests (conference organisation) with Laidet Medical/Asdia, Vitalaire, ALLP, Agiradom and Lowenstein., (Copyright ©The authors 2023.)

Background: Sarcoidosis is a multisystemic disease with a heterogenous course of disease. Comprehensive information about the complexity and treatment indications is essential for improving patient knowledge and adhering to therapy., Objectives: The aim of our study was to investigate the level and resources of information in patients with sarcoidosis and to analyze differences in patient subgroups including age and gender., Methods: We conducted a questionnaire-based online survey in Germany and three semi-structured focus group interviews. The interviews were evaluated independently by two investigators using a structured qualitative content analysis., Results: A total of 402 completed questionnaires were analyzed, 65.8% of participants were women, and the mean age was 53 years. The majority of patients felt well informed about their disease in general (59.4%), but 40.6% were inadequately informed. The most relevant information gaps related to the future perspective (70.6%) as well as fatigue and diffuse pain (63.9%). Most patients received information from their treating pulmonologist (72.1%). 94% used the internet, especially homepages of patient support groups (75.2%). Male participants more often reported being well informed about their disease and were more satisfied with the information (p = 0.001). During the interviews, patients expressed their wish for more comprehensive information and highlighted the importance of psychological co-care as well as the future perspective., Conclusions: A relevant proportion of patients with sarcoidosis are inadequately informed about their own disease, particularly with regard to factors impeding quality of life such as fatigue. Efforts are needed to improve the level and quality of information., (© 2023 S. Karger AG, Basel.)

Background: As fibrosing interstitial lung diseases (fILDs) are associated with high mortality, monitoring of disease activity under treatment is highly relevant. Krebs von den Lungen-6 (KL-6) is associated with the presence and severity of different fILDs, mainly in Asian patient populations., Objectives: Our aim was to evaluate KL-6 as a predictive biomarker in fILDs in Caucasian patients., Methods: Consecutive patients with fILDs were recruited prospectively and serum concentrations of KL-6 were measured at baseline (BL), after 6 and 12 months (6 Months, 12 Months). Clinical characteristics including pulmonary function tests were assessed at 6-monthly visits and correlated with KL-6 BL levels as well as with KL-6 level changes., Results: A total of 47 fILD patients were included (mean age: 65 years, 68% male). KL-6 levels at BL were significantly higher in fILD patients than in healthy controls (n = 44, mean age: 45, 23% male) (ILD: 1,757 ± 1960 U/mL vs. control: 265 ± 107 U/mL, p &lt; 0.0001). However, no differences were noted between ILD subgroups. KL-6 decreased significantly under therapy (6M∆BL-KL6: -486 ± 1,505 mean U/mL, p = 0.032; 12M∆BL-KL6: -547 ± 1,782 mean U/mL, p = 0.041) and KL-6 level changes were negatively correlated with changes in pulmonary function parameters (forced vital capacity [FVC]: r = -0.562, p &lt; 0.0001; DLCOSB: r = -0.405, p = 0.013). While neither absolute KL-6 levels at BL nor KL-6 level changes were associated with ILD progression (FVC decline ≥10%, DLCOSB decline ≥15% or death), patients with a stable FVC showed significantly decreasing KL-6 levels (p = 0.022)., Conclusions: A decline of KL-6 under therapy correlated with a clinically relevant stabilization of lung function. Thus, KL-6 might serve as a predictive biomarker, which however must be determined by larger prospective cohorts., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

An interstitial lung disease represents a relevant organ manifestation in many systemic rheumatic diseases (connective tissue disease-interstitial lung disease, CTD-ILD). In 10% of the cases pulmonary fibrosis even results in an underlying systemic disease. The CTD-ILDs are frequently associated with a poor prognosis. Therefore, it is important to test patients with systemic rheumatic diseases timely and regularly for the presence of an ILD. Treatment decisions should be made together with pneumologists and rheumatologists, particularly with respect to the initiation of a specific treatment. Treatment is based on randomized studies only in a few cases and can mostly be derived from case control studies. For systemic sclerosis-associated ILD (SSc-ILD) antifibrotic treatment with nintedanib has also now been approved in addition to an immunosuppressive treatment. For other CTD-ILDs an antifibrotic treatment should be discussed in an interdisciplinary approach depending on the underlying disease corresponding to a progressively fibrosing ILD., (© 2021. Springer Medizin Verlag GmbH, ein Teil von Springer Nature.)