ERS statement on transition of care in childhood interstitial lung diseases.

Interstitial lung diseases (ILD) are a heterogeneous group of rare diffuse diseases affecting the lung parenchyma in children and adults. Childhood interstitial lung diseases (chILD) are often diagnosed at very young age, affect the developing lung, and can have different presentations and prognosis compared to adult forms of these diseases. In addition, chILD in many cases may apparently remit, and have a better response to therapy and better prognosis than adult ILD.

Expression of PD-1/PD-L1 axis in mediastinal lymph nodes and lung tissue of human and experimental lung fibrosis indicates a potential therapeutic target for idiopathic pulmonary fibrosis.

Background: Mediastinal lymph node enlargement is prevalent in patients with idiopathic pulmonary fibrosis (IPF). Studies investigating whether this phenomenon reflects specific immunologic activation are lacking.

Methods: Programmed cell death-1 (PD-1)/ programmed cell death ligand-1 (PD-L1) expression in mediastinal lymph nodes and lung tissues was analyzed.

ABCA3-related interstitial lung disease beyond infancy.

Background: The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of life and succumb to disease if not lung transplanted. This register-based cohort study reviews patients with ABCA3 lung disease who survived beyond the age of 1 year.

Method: Over a 21-year period, patients diagnosed as chILD due to ABCA3 deficiency were identified from the Kids Lung Register database.

Psoriatic Insomnia: A Subjective and Objective Sleep Evaluation.

Psoriasis may affect patients' sleep. In order to examine this relationship, this study evaluated non-anxious and non-depressive patients with moderate to severe psoriasis before and after 6 months of systemic treatment. A prospective case-control study with 46 consecutive patients (mean age 51.

Lung cancer in patients with idiopathic pulmonary fibrosis: A retrospective multicentre study in Europe.

Background And Objective: There remains a paucity of large databases for patients with idiopathic pulmonary fibrosis (IPF) and lung cancer. We aimed to create a European registry.

Methods: This was a multicentre, retrospective study across seven European countries between 1 January 2010 and 18 May 2021.

Sestrin 2 levels are associated with emphysematous phenotype of COPD.

Sestrins (Sesns) are a family of highly conserved stress-inducible proteins and various stresses have been shown to strongly up-regulate them. Sestrin 2 (Sesn2) deficiency has been shown to partially suppress pulmonary emphysema. The aim of this study was to evaluate Sesn2 levels in COPD patients and its possible associations with the presence of emphysema and blood eosinophils.

Paucigranulocytic Asthma: Potential Pathogenetic Mechanisms, Clinical Features and Therapeutic Management.

Asthma is a heterogeneous disease usually characterized by chronic airway inflammation, in which several phenotypes have been described, related to the age of onset, symptoms, inflammatory characteristics and treatment response. The identification of the inflammatory phenotype in asthma is very useful, since it allows for both the recognition of the asthmatic triggering factor as well as the optimization of treatment The paucigranulocytic phenotype of asthma (PGA) is characterized by sputum eosinophil levels <1−3% and sputum neutrophil levels < 60%. The precise characteristics and the pathobiology of PGA are not fully understood, and, in some cases, it seems to represent a previous eosinophilic phenotype with a good response to anti-inflammatory treatment.

Reduced immunogenicity of the mRNA vaccine BNT162b2 in patients with idiopathic pulmonary fibrosis.

https://bit.ly/3K2KXQ0.

Long-term efficacy and safety of omalizumab in patients with allergic asthma: A real-life study.

The efficacy and safety of omalizumab in patients with severe allergic asthma have been established in both randomized controlled trials and real-life studies. To evaluate the sustained effectiveness and safety of long-term treatment with omalizumab in a real-world setting. In this retrospective study, we included patients treated with omalizumab for at least 8 years in four asthma clinics in Greece.

Device use errors among patients with asthma and COPD and the role of training: a real-life study.

: Administration of inhaled medication for asthma and COPD is often difficult and incorrect device use is associated with unfavorable outcomes. We aimed to evaluate device use errors in asthma and COPD patients and to associate incorrect use with the patient's characteristics and medical history.: Demographics and medical history were recorded.

Switching between biologics in severe asthma patients. When the first choice is not proven to be the best.

During the last decades, new treatments targeting disease mechanisms referred as biologics have been introduced in the therapy of asthma and currently, five monoclonal antibodies have been approved. Although these therapeutic agents have been formulated to target specific asthma endotypes, it is often difficult for the treating physician to identify which patient is the best candidate for each one of these specific treatments especially in the clinical scenario of a patient in whom clinical characteristics overlap between different endotypes, allowing the selection of more than one biologic agent. As no head-to-head comparisons between these biologics have been attempted, there is no evidence on the superiority of one biologic agent over the other.

Economic Burden and Management of Systemic Sclerosis-Associated Interstitial Lung Disease in 8 European Countries: The BUILDup Delphi Consensus Study.

Introduction: Systemic sclerosis (SSc) is a rare chronic autoimmune disease characterised by microvascular damage, immune dysregulation and fibrosis, affecting the skin, joints and internal organs. Interstitial lung disease (ILD) is frequently associated with systemic sclerosis (SSc-ILD), leading to a poor prognosis and a high mortality rate. The aim of the BUILDup study (BUrden of Interstitial Lung Disease Consensus Panel) was to investigate the overall disease management and to estimate the social and economic burden of SSc-ILD across 8 European countries.

The Burden of Progressive Fibrosing Interstitial Lung Disease: A DELPHI Approach.

Introduction: The term progressive fibrosing interstitial lung disease (ILD) describes patients with fibrotic ILDs who, irrespective of the aetiology of the disease, show a progressive course of their disease despite current available (and non-licensed) treatment. Besides in idiopathic pulmonary fibrosis, little is known about management and the burden of patients with fibrotic ILD, particularly those with a progressive behaviour.

Methods: Using the Delphi method, 40 European experts in ILD management delivered information on management of (progressive) fibrosing ILD and on the impact of the disease on patients' quality of life (QoL) and healthcare resource utilisation (HCRU).

Sirtuin 1: Endocan and Sestrin 2 in Different Biological Samples in Patients with Asthma. Does Severity Make the Difference?

Background: Sestrin 2, Endocan, and Sirtuin 1 are distinct molecules with some biologic actions associated with asthma pathophysiology. The aim of the present study was to determine the molecular level differences attributable to underlying asthma severity.

Methods: We initially recruited 85 asthmatics with a wide spectrum of severity.

Prospective evAluatIon foR inhalation devices in Greek patients with COPD and asthma: The PAIR study.

Introduction: Chronic obstructive pulmonary disease (COPD) and asthma remain a major health burden. Adherence to inhaled therapy is critical in order to optimize treatment effectiveness. Properly designed questionnaires can assess patients' satisfaction with their inhaler devices.

Lung cancer in patients with Idiopathic Pulmonary Fibrosis. A retrospective multicenter study in Greece.

Background: Abundant evidence supports an association between Idiopathic Pulmonary Fibrosis (IPF) and lung cancer development. Data on diagnosis and management of patients with IPF and lung cancer are still scarce.

Patients And Methods: This was a retrospective multicenter study, enrolling 1016 patients with IPF from eight different centers between 2011 and 2018 in Greece.

Idiopathic Pulmonary Fibrosis: Best Practice in Monitoring and Managing a Relentless Fibrotic Disease.

Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease that is, by definition, progressive. Progression of IPF is reflected by a decline in lung function, worsening of dyspnea and exercise capacity, and deterioration in health-related quality of life. In the short term, the course of disease for an individual patient is impossible to predict.

Existing and emerging treatments for idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a progressive and invariable fatal interstitial lung disease. Current antifibrotic treatment halts disease progression but does not cure the disease itself. In the last decade, a substantial understanding of disease pathobiological mechanisms led to the development of numerous clinical trials testing promising pharmacologic agents.

Serum osteopontin in patients with lung cancer and chronic obstructive pulmonary disease: does the co-existence make the difference?

Background: Osteopontin (OPN) is involved in cancer development and metastasis. Increased sputum OPN was detected in chronic obstructive pulmonary disease (COPD).

Methods: We evaluated serum OPN levels in patients with lung cancer (LC) and/or COPD and aimed to determine OPN prognostic performance in 1-year mortality in LC and also its diagnostic performance in LC among COPD patients.