ERS International Congress 2023: highlights from the Interstitial Lung Diseases Assembly.

This article summarises a selection of scientific highlights in the field of interstitial lung diseases (ILDs) presented at the International Congress of the European Respiratory Society in 2023. Translational and clinical studies focused on the whole spectrum of ILDs, from (ultra)rare ILDs to sarcoidosis, ILDs associated with connective tissue disease and idiopathic pulmonary fibrosis. The main topics of the 2023 Congress presentations were improving the diagnostic process of ILDs, better prediction of disease course and investigation of novel treatment options.

Study protocol for connective tissue disease-associated interstitial lung disease trial (TEL-CTD-ILD): A randomized controlled trial of a home-based telemonitoring of treatment effects.

INTRODUCTION: Interstitial lung disease is one of the most severe pulmonary complications related to connective tissue diseases, resulting in substantial morbidity and mortality. Telepneumology has the potential to improve the long-term management of patients with CTD-ILD. We propose a randomized controlled trial to evaluate the efficacy of home-based telemonitoring of patients with CTD-ILD, in whom treatment was initiated. MATERIALS AND METHODS: We will conduct a randomized controlled trial comparing the standard of care with a telemonitoring program. Telemonitoring will start 10 to 14 days before treatment and will be carried out for three months of therapy. After initial training, patients from the intervention group will perform daily spirometry (FVC), transdermal pulse oximetry, pulse and blood pressure measurements, activity measurement (accelerometry), and assessment of the severity of cough and dyspnea. The results will be reported using a telemetric system designed by Mediguard® for this study. The primary outcome measure will be the health-related quality of life change using EQ-5D-5L questionnaire and St. George's Respiratory Questionnaire, as measured at stationary visits in both study groups. Secondary outcomes will include assessment of lung function, costs of health service utilization, satisfaction from being telemonitored, dyspnea by mMRC, fatigue by FAS, patients' adherence to recommended medications using the ASCD, anxiety and depression symptoms as measured by HADS, PHQ-9, and side effects of treatment. DISCUSSION: This is the first clinical trial protocol to evaluate home-based telemonitoring to optimize connective tissue disease-associated interstitial lung management. The study aims to provide data on the impact of telemonitoring on quality of life, evaluation of health status of patients with CTD-ILD using telemonitoring versus standard care. Additionally, we will evaluate the cost-effectiveness of telemonitoring solutions in patients with CTD-ILD. TRIAL REGISTRATION: ClinicalTrials.gov Identifier: NCT04428957; Registered June 11, 2020; https://clinicaltrials.gov/ct2/show/NCT04428957.

Patterns of lung fibrosis in patients with interstitial pneumonia with autoimmune features and connective tissue diseases-associated interstitial lung disease-a narrative review.

BACKGROUND AND OBJECTIVE: Interstitial pneumonia with autoimmune features (IPAF) was defined in 2015 as a research statement of European Respiratory Society and American Thoracic Society. Connective tissue diseases (CTDs) manifest in the respiratory tract, the main manifestation being interstitial lung disease, which contributes to morbidity and mortality. This poses numerous clinical challenges and is a substantial burden on healthcare systems around the world. The objective of this narrative review was to provide readers with a comprehensive and extensive overview of such manifestations in the respiratory system, analysis of prevalence of specific manifestations in the lung and to characterize population of patients with IPAF. METHODS: We reviewed the current state of knowledge on radiological findings in interstitial lung diseases in course of convective tissue diseases and IPAF, a narrative review of PubMed database using Advanced Search Builder was performed. The search was conducted from 15.07.2021 to 03.04.2022. A total of 655 articles in English were reviewed. KEY CONTENT AND FINDINGS: Similarities and differences between interstitial pneumonia in course of well-established CTDs and IPAF are discussed with special emphasis on required future research areas. Manifestations of CTDs in the respiratory system are overviewed, with the emphasis on interstitial lung disease, as despite clinical similarities of various connective tissue diseases, there is variability in their presentation in the respiratory system, radiological patterns and clinical outcomes. We would also like to draw readers' attention to clinical significance of interstitial lung abnormality both in the context of CTDs and lack of underlying autoimmune disorders. CONCLUSIONS: There is need for prospective cohort studies regarding the natural course of IPAF, its clinical stability and its manifestations in the respiratory system. Prospective studies are also required to evaluate diagnostic and prognostic factors of IPAF. Interstitial lung disease associated with CTDs (CTD-ILD) is a significant factor impacting clinical outcomes and patient prognosis, therefore its diagnostic work-up is best performed by an experienced multidisciplinary team and with use of procedures of high diagnostic yield.

Review: Serum Biomarkers of Lung Fibrosis in Interstitial Pneumonia with Autoimmune Features-What Do We Already Know?

Interstitial pneumonia with autoimmune features (IPAF) belongs to a group of diseases called interstitial lung diseases (ILDs), which are disorders of a varied prognosis and course. Finding sufficiently specific and sensitive biomarkers would enable the progression to be predicted, the natural history to be monitored and patients to be stratified according to their treatment. To assess the significance of pulmonary fibrosis biomarkers studied thus far, we searched the PubMed, Medline and Cochrane Library databases for papers published between January 2015 and June 2021. We focused on circulating biomarkers. A primary review of the databases identified 38 articles of potential interest. Overall, seven articles fulfilled the inclusion criteria. This review aims to assess the diagnostic and prognostic value of molecules such as KL-6, SP-A, SP-D, circulating fibrocytes, CCL2, CXCL13, CXCL9, CXCL10 and CXCL11. All of these biomarkers have previously been studied in idiopathic pulmonary fibrosis (IPF) and connective tissue disease-associated interstitial lung disease (CTD-ILD). IPAF is a disorder of a heterogeneous nature. It explains the lack of coherent observations in terms of correlations with functional parameters. There is still no meta-analysis of pulmonary fibrosis biomarkers in IPAF. This is mainly due to the heterogeneity of the methodology and groups analysed in the research. More research in this area is needed.