National survey on the presence of multidisciplinary meetings for interstitial lung diseases (ILD-MDM) in hospitals in Germany.

Background: Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking.

ERS/EULAR clinical practice guidelines for connective tissue diseases associated interstitial lung disease.

Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care.

Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies.

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung diseaseDeveloped by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR)Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).

Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care.

Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies.

Osteoprotegerin is elevated in pulmonary fibrosis and associates with IPF progression: a pilot study.

Background Osteoprotegerin (OPG), a decoy receptor for receptor activator of NF-kB ligand (RANKL), serves as a biomarker for liver fibrosis severity. Our recent findings show OPG production in fibrotic lung tissue, though its specific cellular source and role in pulmonary fibrosis are unknown. We hypothesized that OPG is produced by fibroblasts and serves as a marker for pulmonary fibrosis progression.

[National survey on the presence of multidisciplinary meetings for interstitial lung diseases (ILD-MDM) in hospitals in Germany].

Background:  Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking.

[National survey on the presence of multidisciplinary meetings for interstitial lung diseases (ILD-MDM) in hospitals in Germany. German version].

Background: Interstitial lung diseases (ILD) represent an interdisciplinary clinical challenge and are not uncommonly associated with rheumatological diseases. Interstitial lung disease multidisciplinary meetings (ILD-MDM) provide a structured platform for interdisciplinary case discussions and decision making. Despite their great importance in patient care, data on the prevalence, structure and function of ILD-MDM in Germany are lacking.

Treating connective tissue disease-associated interstitial lung disease - think outside the box: a perspective.

Connective tissue disease-associated interstitial lung disease is one of the most common subtypes of interstitial lung disease, which is a leading cause of morbidity and mortality in patients with these systemic autoimmune rheumatic diseases. A spectrum of disease trajectories exists within individual and across different connective tissue diseases. In individuals with connective tissue diseases who are at risk or at the early asymptomatic stage with interstitial lung changes, we have potential windows of opportunity for interventions to prevent the development of or progression to interstitial lung disease.

Accelerate your career: the power of the ERS interstitial lung disease mentorship programme.

https://bit.ly/4iGBr6G.

Image analysis: Ga-FAPI-46 PET derived texture parameters improve the differentiation of malignant and benign pulmonary lesions.

Background: Pulmonary lesions inconclusive in F-FDG PET/CT are a known clinical problem. Both texture analysis and Ga-FAPI-46 have shown potential in thoracic oncological problems but their combination has not been assessed yet. This initial analysis aims to evaluate the utility of Ga-FAPI-46 PET texture parameters to differentiate between lung cancer and benign pulmonary lesions inconclusive in F-FDG PET/CT.

Real-life characteristics and management of patients with fibrosing interstitial lung disease: INSIGHTS-ILD registry.

Background: This study aims to report real-life data on the characteristics and treatment patterns of patients with fibrosing interstitial lung disease (ILD; except idiopathic pulmonary fibrosis) across multiple specialised centres in Germany. Eligibility criteria included ILD affecting >10% of lung parenchyma on high-resolution computed tomography, a single breath diffusion capacity for carbon monoxide ( ) ≤80% predicted and active treatment of lung disease.

Methods: As of the interim analysis cut-off, 655 patients (mean±sd age 65.

Nerandomilast in Patients with Progressive Pulmonary Fibrosis.

Background: Nerandomilast (BI 1015550) is an orally administered preferential inhibitor of phosphodiesterase 4B with antifibrotic and immunomodulatory properties. Nerandomilast has been shown to slow the progression of idiopathic pulmonary fibrosis, but an assessment of its effects in other types of progressive pulmonary fibrosis is needed.

Methods: In a phase 3, double-blind trial, we randomly assigned patients with progressive pulmonary fibrosis in a 1:1:1 ratio to receive nerandomilast at a dose of 18 mg twice daily, nerandomilast at a dose of 9 mg twice daily, or placebo, with stratification according to background therapy (nintedanib vs.

Nerandomilast in Patients with Idiopathic Pulmonary Fibrosis.

Background: Nerandomilast (BI 1015550) is an orally administered preferential inhibitor of phosphodiesterase 4B with antifibrotic and immunomodulatory effects. In a phase 2 trial involving patients with idiopathic pulmonary fibrosis, treatment with nerandomilast stabilized lung function over a period of 12 weeks.

Methods: In this phase 3, double-blind trial, we randomly assigned patients with idiopathic pulmonary fibrosis in a 1:1:1 ratio to receive nerandomilast at a dose of 18 mg twice daily, nerandomilast at a dose of 9 mg twice daily, or placebo, with stratification according to background antifibrotic therapy (nintedanib or pirfenidone vs.

Effect of Admilparant, a Lysophosphatidic Acid Receptor 1 Antagonist, on Disease Progression in Pulmonary Fibrosis.

Background: Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are chronic fibrosing interstitial lung diseases associated with irreversible loss of lung function and early mortality. Admilparant (BMS-986278) is an oral lysophosphatidic acid receptor 1 antagonist under development for treatment of IPF and PPF.

Research Question: How does admilparant affect time to disease progression in patients with IPF or PPF?

Study Design And Methods: In a phase 2, randomized, double-anonymized, placebo-controlled study, parallel cohorts of patients with IPF or PPF were randomized separately 1:1:1 to receive 30 mg admilparant, 60 mg admilparant, or placebo twice daily for 26 weeks; background antifibrotics were allowed.

Sex-specific aspects in a population of patients undergoing evaluation for interstitial lung disease with transbronchial cryobiopsy.

There are well-documented differences in idiopathic pulmonary fibrosis (IPF) between sexes. The sex-specific prevalence of interstitial lung disease (ILD) subtypes in patients who require a full diagnostic work-up, including transbronchial cryobiopsy (TCB), after initial multidisciplinary discussion (MDD) is still unknown. Retrospective analysis of sex dispareties in patients with ILD who received an interdisciplinary indication for lung biopsy and underwent bronchoalveolar lavage, TCB and, if necessary, surgical lung biopsy at our ILD centre in Heidelberg between 11/17 and 12/21.

ERS Congress 2024: highlights from the Interstitial Lung Diseases Assembly.

https://bit.ly/3WAhw19.

Person-centred health outcomes in the routine care for people with progressive pulmonary fibrosis: the COCOS-IPF project's European survey on healthcare professionals' views and practices.

Background: Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are interstitial lung diseases (ILD) that carry a high burden and mortality. IPF/PPF experts and patients call for standardized care, outcome harmonization and holistic management in these complex and devastating diseases, with a focus on person-centeredness. In this cross-sectional international survey study, we aimed to gather information on the person-centred health outcomes European healthcare professionals (HCPs) already use or deem important for use in routine care for IPF/PPF.

Late-onset telomere biology disorders in adults: clinical insights and treatment outcomes from a retrospective registry cohort.

Pathogenic germ line variants affecting proper telomere maintenance result in premature telomere shortening and cause telomere biology disorders (TBDs). Although classical dyskeratosis congenita in children is rather well defined, late-onset ("cryptic") TBDs remain underrecognized, resulting in underdiagnosis and inadequate treatment in affected adults. Here, we present a series of adult TBD cases collected through the German TBD reference center between 2014 and 2024.

Diagnosis, screening, and follow-up of patients with familial interstitial lung disease: Results from an international survey.

Background: Advances in the field of genetics of interstitial lung diseases (ILDs) have led to the recent consensus statements made by expert groups. International standards for genetic testing in ILD have not yet been established. We aimed to examine current real-world strategies employed by pulmonologists working with familial ILD.

Diagnosis and Treatment of Hypersensitivity Pneumonitis: S2k Guideline of the German Respiratory Society and the German Society for Allergology and Clinical Immunology.

German recommendations for the diagnosis of hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis, were last published in 2007. The current S2k Guideline for the Diagnosis and Treatment of Hypersensitivity Pneumonitis replaces these diagnostic recommendations. They were supplemented by the aspect of chronic, and in particular of the chronic fibrotic phenotype of HP, and also, as the first HP guideline, include treatment recommendations.

Automated AI-based image analysis for quantification and prediction of interstitial lung disease in systemic sclerosis patients.

Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with rapidly evolving interstitial lung disease (ILD), driving its mortality. Specific imaging-based biomarkers associated with the evolution of lung disease are needed to help predict and quantify ILD.

Methods: We evaluated the potential of an automated ILD quantification system (icolung) from chest CT scans, to help in quantification and prediction of ILD progression in SSc-ILD.

Identifying outcome domains to establish a core outcome set for progressive pulmonary fibrosis: a scoping review.

Introduction: People with idiopathic pulmonary fibrosis (IPF) and other forms of progressive pulmonary fibrosis (PPF) have a high symptom burden and a poor health-related quality of life (HRQoL). Despite efforts to offer specialised treatment, clinical care for these patients remains suboptimal and several nonmedical needs remain unaddressed. Developing a core outcome set (COS) can help to identify a minimum set of agreed-upon outcomes that should be measured and acted-upon in clinical care.

SARS-CoV-2 Vaccination in Primary Humoral Immunodeficiency: Experience from a German Lung Clinic.

Introduction: During the COVID-19 pandemic, the effectiveness of vaccines against SARS-CoV-2 in immunodeficient patients not only did affect the individual risk of these vulnerable patients but endangered the selection of new variants of concern due to prolonged virus shedding by these patients.

Methods: In a tertiary center for pulmonary diseases, we investigated the immune response of 11 patients with primary humoral immunodeficiency and 13 healthy controls on the humoral and cellular level after full vaccination with an mRNA or vector vaccine against SARS-CoV-2.

Results: In the majority of patients (73%), we found antibodies against the spike protein above the threshold of positivity.

Bexotegrast in people with idiopathic pulmonary fibrosis (IPF): a plain language summary of publication of the INTEGRIS-IPF study.

This plain language summary shares results from a clinical study called INTEGRIS-IPF that was published in the in 2024. This study looked at a medicine called (beck-so-teh-grast) as a possible treatment for (i-dee-uh-pa-thick pul-muh-ner-ee fie-bro-sis; IPF). is an investigational medicine, which means that it is being studied and has not yet been approved by the US Food and Drug Administration (FDA), for people with IPF to take as a treatment.