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.

Efficacy and safety of rituximab and low dosage of glucocorticoids for ANCA associated vasculitis interstitial lung disease: a proof-of-concept study.

Introduction: The prevalence of interstitial lung disease (ILD) in ANCA associated vasculitis (AAV) ranges from 13 % to 45 %, and both its definition and management represent a quandary. The occurrence of lung fibrosis and ANCA positivity, particularly in subjects without any other evidence of vasculitis, is far from being clearly interpreted, as well as its proper therapeutic management. In this regard, aim of this study is to assess whether Rituximab (RTX) in association with a low dosage of glucocorticoids (GCs), could be effective also in the treatment of AAV-ILD.

Strategies for Maximising Lung Utilisation in Donors After Brain and Cardiac Death: A Narrative Review.

Lung transplantation remains the standard of care for end-stage lung disease, yet a persistent gap exists between donor lung availability and growing clinical demand. Expanding the donor pool and optimising donor lung management are therefore critical priorities. However, no universally accepted management protocols are currently in place.

Perception of the role of Telemedicine in Interstitial Lung Diseases: -Findings from Società Italiana di Pneumologia/ Italian Respiratory -Society (SIP-IRS) survey.

Background: Telemedicine (TM) is increasingly recognised as a valuable tool in the management of interstitial lung diseases (ILDs). Despite its potential, its integration and application still remain limited. Our work aimed to assess pulmonologists' (physicians and trainees) perception regarding the use of TM in ILDs management.

Serum ferritin as a specific biomarker of anti-MDA5-interstitial lung disease: a multicenter, case-control study in observational and validation datasets.

Background: Serum ferritin has been proposed as biomarker of interstitial lung disease (ILD) in anti-MDA5 dermatomyositis (DM). Nevertheless, no data exist for serum ferritin in other idiopathic inflammatory myopathies (IIM)-ILD nor in IIM without ILD. Aim of this case-control study was to assess whether serum ferritin may be considered a specific and sensitive biomarker for IIM-ILD, as well as to assess whether it correlates with clinical and immunological findings.

Electronic Nicotine Delivery Systems (ENDS): Implications for the Clinician.

The evidence that tobacco cigarettes are harmful to the health of smokers led to the introduction of electronic nicotine delivery systems (ENDS) as a safer alternative. ENDS, which include electronic cigarettes (e-cigs) and heated tobacco products (battery-operated devices that heat a liquid and produce an aerosol), are portable, cheap, easy-to-use, self-powered devices, and resemble tobacco cigarettes. After an overview of the toxicological, clinical, and epidemiological implications associated with the increasingly widespread use of ENDS, this narrative paper evaluates their role as a smoking cessation aid.

Effectiveness of Nintedanib in Progressive Pulmonary Fibrosis Assessed by Progression Criteria: An Italian, Observational, Multicenter Study.

Purpose: Pulmonary fibrosis is defined as progressive based on the combination of radiological, clinical, and functional criteria. Nintedanib can reduce the rate of lung function decline, but no data are available on its effectiveness to hamper disease progression evaluated by these criteria. The primary aim of the study was to assess the number of patients with progressive pulmonary fibrosis (PPF) who no longer meet progression criteria after one year of treatment with nintedanib.

Allogeneic Hematopoietic Stem Cell Transplantation Despite Severe Infection in a Lung Transplanted Patient-A Case Report.

Solid-organ transplant patients require prolonged immunosuppression, increasing their risk of hematologic disorders. For these conditions, allogeneic hematopoietic stem cell transplantation (HSCT) is a potential treatment, but it carries significant risk of treatment-related mortality due to the high possibility of developing rare infectious complications. We report a case of a 55-years-old male with a history of bilateral lung transplantation for extrinsic allergic alveolitis in 2015, who developed acute myeloid leukemia/myelodysplastic syndrome (AML/MDS) with TP53 mutation seven years later.

Donor-Recipient Mismatch in Lung Transplantation: The Role of Graft Sizing in Clinical Outcomes.

Lung transplantation is a life-saving procedure for end-stage lung diseases. Size matching is critical in the donor-recipient selection process. This retrospective study analyzed 146 patients who underwent lung transplantation between 2013 and 2023.

A novel web-based tool for lung transplant patients undergoing extracorporeal photopheresis.

Background: Extracorporeal photopheresis (ECP) is considered an emerging rescue therapy for patients with chronic lung allograft dysfunction (CLAD). The aim of the study was to set up a web-based data collection tool for lung transplant patients with CLAD undergoing ECP.

Methods: The web-based tool was developed using Oracle MySQL and coded in HyperText Markup Language, JavaScript and Cascading Style Sheets and was set up with pre- and post-transplant data of possible interest in CLAD.

Objective Effects and Patient Preferences for Ambulatory Oxygen in Fibrotic Interstitial Lung Disease With Isolated Exertional Hypoxaemia: A Placebo-Controlled 6-Minute Walk Test Study.

Background And Objective: The available evidence on the effects of ambulatory oxygen on exercise impairment in patients with fibrotic interstitial lung diseases (F-ILD) is of limited quality.

Methods: We conducted a randomised, double-blind, placebo-controlled crossover trial with 32 normoxaemic F-ILD patients, desaturating to ≤ 88% during a baseline 6-minute walk test (6MWT) on ambient air. After determining the oxygen flow needed to prevent desaturation, patients completed two double-blind 6MWTs with either oxygen or placebo (compressed medical air) at the same personalised flow.

Prevalence and Predictors of Response to Antifibrotics in Long-Term Survivors with Idiopathic Pulmonary Fibrosis.

Purpose: The natural history of IPF remains unpredictable despite antifibrotic treatment. In addition, some patients discontinue treatment due to the occurrence of adverse events. To date, no data exist on either the effect of long-term treatment or predictors of treatment response.

Spirotetrahydroisoquinoline-Based Histone Deacetylase Inhibitors as New Antifibrotic Agents: Biological Evaluation in Human Fibroblasts from Bronchoalveolar Lavages of Idiopathic Pulmonary Fibrosis Patients.

Idiopathic pulmonary fibrosis (IPF) is a rare interstitial lung disease typified by a progressive fibrosing phenotype. IPF has been associated with aberrant HDAC activity, particularly HDAC6. Combining synthetic and modeling studies, a new family of spirotetrahydroisoquinoline-capped histone deacetylase inhibitors - was developed.

CSF IL-6, GDF-15, GFAP and NfL levels in early Alzheimer disease: a pilot study.

Background: Despite their potential usefulness as biomarkers, no study has investigated the interactions between cerebrospinal fluid (CSF) changes of neurofilament light chain (NfL), glial fibrillary acidic protein (GFAP), growth differentiation factor 15 (GDF-15), transactive response DNA binding protein (TDP-43) and interleukin-6 (IL-6) and the core AD CSF biomarkers in the same cohort of AD patients.

Objectives: The aim of this pilot study is to evaluate the CSF levels of these analytes in patients with AD and assess their clinical relevance in this neurological condition.

Design: Cross-sectional study.

Computed tomography in severe asthma assessment: a systematic review.

Objective: Chest computed tomography (CT) is usually performed in patients with severe asthma (SA) to exclude concomitant conditions related to poor clinical control. Despite the growing evidence regarding the utility of CT in the characterization of morphological abnormalities and airway remodeling, its role in SA assessment is still largely unexplored. The aim of our systematic review was to evaluate published data investigating the role of chest CT in patients with SA.

The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry.

Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.

Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.

Regulatory T Cell Phenotype Related to Cytokine Expression Patterns in Post-COVID-19 Pulmonary Fibrosis and Idiopathic Pulmonary Fibrosis.

Background: Post-coronavirus disease 19 lung fibrosis (PCLF) shares common immunological abnormalities with idiopathic pulmonary fibrosis (IPF), characterized by an unbalanced cytokine profile being associated with the development of lung fibrosis. The aim of the present study was to analyze and compare the different subsets of CD4- and CD8-T cells, along with specific cytokine expression patterns, in peripheral blood (PB) from patients affected by PCLF and IPF and healthy controls (HCs).

Methods: One-hundred patients followed at the Rare Lung Disease Center of Siena University Hospital were enrolled.

Pre-biologic FeNO might predict anti-IL-5/IL-5Rα response to treatment in severe asthmatics.

Objective: It remains unclear whether baseline FeNO levels can predict response to anti-IL5/5R biologic treatment in patients with severe asthma.

Methods: We recruited 104 patients with severe eosinophilic asthma treated with anti-IL5/anti-IL5R for at least one year who had measured FeNO values before the beginning of anti-eosinophilic treatment. Population was divided into subjects with FeNO < 25 and ≥25 ppb.

Online survey on existing sarcoidosis registries and biobanks: an ERN lung initiative.

The present survey, promoted by the European Reference Network on rare respiratory diseases (ERN-Lung, Core Networks Sarcoidosis and ILD), aims to assess the existing sarcoidosis registries and biobanks across Europe and to compare the various types of biospecimen collected, the different procedures performed, and the sample storage conditions applied. This survey was initiated by the European Reference Network on rare respiratory diseases (ERN-Lung) Core Network "Sarcoidosis" in April 2023. The survey was launched by ERN-Lung Core Network "Sarcoidosis" in August 2023 and remained active until end of February 2024.

Respiratory Trajectories and Correlation with Serum Biochemical Indices in Spinal and Bulbar Muscular Atrophy.

Background/objectives: The primary life-threatening complication in spinal-bulbar muscular atrophy (SBMA) is ventilatory failure. The present study analyzes the longitudinal patterns of respiratory function tests over a follow-up of 11 years.

Methods: We collected data from 9 genetically confirmed SBMA patients.

Efficacy and safety of a step-down regimen of low dosage of glucocorticoids combined with early administration of synthetic or biologic immunosuppressants in anti-synthetase syndrome: A pilot study.

Introduction: Anti-synthetase syndrome (ASS) is a rare autoimmune disease characterized by the presence of anti-aminoacyl-transfer-RNA synthetase antibodies (ARS) and the involvement of muscles, skin, joints, and lungs. Despite increasing interest and evidence, optimal clinical management remains unclear due to a lack of randomized control trials. This study aims to evaluate the efficacy and safety of a treatment regimen involving early co-administration of glucocorticoids and immunosuppressants, with rapid prednisone tapering.