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.

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.

Natural killer cells in the lung: novel insight and future challenge in the airway diseases.

Natural killer (NK) cells are innate lymphoid cells which are present in the lung as circulating and resident cells. They are key players both in airway surveillance and in crosstalk with (COPD) pathogenesis, and they seem to contribute to the development of bronchiectasis. In asthma, NK cell dysfunction was observed mainly in severe forms, and it can lead to a biased type-2 immune response and failure in the resolution of eosinophilic inflammation that characterise both allergic and eosinophilic phenotypes.

Thoracic Malignancies: From Prevention and Diagnosis to Late Stages.

Lung cancer remains the leading cause of cancer-related mortality worldwide, characterized by its complexity and heterogeneity [...

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.

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.

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.

Benralizumab: from tissue distribution to eosinophilic cytotoxicity up to potential immunoregulation.

Introduction: Benralizumab, a monoclonal IgG antibody, has emerged as a key therapeutic agent in severe asthma by specifically targeting eosinophils, pivotal cells that drive inflammation and tissue damage. Over the past two decades, the availability of such targeted therapies has allowed patients to achieve better disease control. Real-world evidence has consistently demonstrated the effectiveness of benralizumab in managing severe asthma.

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.

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.

Diagnostic Performance of CLEIA Versus FEIA for KL-6 Peripheral and Alveolar Concentrations in Fibrotic Interstitial Lung Diseases: A Multicentre Study.

Background: Interstitial lung diseases (ILD) is a group of lung disorders characterized by interstitial lung thickening due to inflammatory and fibrotic processes. Krebs von den Lungen-6 (KL-6) is a molecule secreted by damaged type II alveolar pneumocytes in the alveolar space. The goal of the present study was to compare two detection methods of KL-6 in both bronchoalveolar lavage (BAL) and serum from ILD patients at the moment of diagnosis.

Krebs von den Lungen-6 as biomarker of the new progressive fibrotic phenotype of interstitial lung disease.

Background: Novel progressive fibrotic phenotype has recently been proposed characterized by progressive and inexorable worsening of the disease. Krebs von den Lungen-6 (KL-6) has been proposed as fibrotic-ILD biomarker. We aimed to assess the role of KL-6 in fibrotic-ILD and the progressive phenotype in accordance with serial serum KL-6.

Evaluation of multiple-flows exhaled nitric oxide and its clinical significance in severe asthmatic patients treated with biologics: a prospective real-life study.

Background: Specific biomarkers, such as eosinophilia in peripheral blood or fractional exhaled nitric oxide (FeNO), can guide us in the choice of biologic therapy, allowing a more personalized approach. Although there are multiple evidences in the literature about the role of FeNO as a predictor of response to different biologic treatments, there are no data on the relationship between FeNO changes and clinical response to the four biologic drugs currently in use.

Objective: To evaluate and to compare the expression of multiple-flows FeNO parameters in a cohort of patients with severe asthma (SA) before and during the treatment with biologics to evaluate the performance of these biomarkers in predicting the achievement of clinical remission.

Altered serum concentrations of IL-8, IL-32 and IL-10 in patients with lung impairment 6 months after COVID-19.

Post-COVID symptoms are reported in 10-35 % of patients not requiring hospitalization, and in up to 80 % of hospitalized patients and patients with severe disease. The pathogenesis of post-COVID syndrome remains largely unknown. Some evidence suggests that prolonged inflammation has a key role in the pathogenesis of most post-COVID manifestations.

SARS-CoV-2 and Dysphagia: A Retrospective Analysis of COVID-19 Patients with Swallowing Disorders.

Background: COVID-19 can lead to impairment of neural networks involved in swallowing, since the act of swallowing is coordinated and performed by a diffuse brain network involving peripheral nerves and muscles. Dysphagia has been identified as a risk and predictive factor for the severest form of SARS-CoV-2 infection.

Objectives: To investigate the association between swallowing disorders and COVID-19 in patients hospitalized for COVID-19.

Immunological Similarities and Differences between Post-COVID-19 Lung Sequelae and Idiopathic Pulmonary Fibrosis.

Introduction: Pulmonary fibrosis is an irreversible condition that may be caused by known (including viral triggers such as SARS-CoV-2) and unknown insults. The latter group includes idiopathic pulmonary fibrosis (IPF), which is a chronic, progressive fibrosing interstitial pneumonia of unknown cause. The longer the insult acts on lung tissue, the lower the probability of a complete resolution of the damage.

Role of BAL and Serum Krebs von den Lungen-6 (KL-6) in Patients with Pulmonary Fibrosis.

Interstitial lung diseases (ILDs) encompass a diverse group of disorders affecting the lung interstitium, leading to inflammation, fibrosis, and impaired respiratory function. Currently, the identification of new diagnostic and prognostic biomarkers for ILDs turns out to be necessary. Several studies show the role of KL-6 in various types of interstitial lung disease and suggest that serum KL-6 levels can be used as a prognostic marker of disease.

Predictive Role of Cytokine and Adipokine Panel in Hospitalized COVID-19 Patients: Evaluation of Disease Severity, Survival and Lung Sequelae.

Coronavirus disease 2019 (COVID-19) may determine a multisystemic chronic syndrome after resolution of SARS-CoV-2 infection in a significant percentage of patients. Persistent cytokine dysregulation can contribute to long-lasting inflammation and tissue damage, resulting in the diverse, often debilitating symptoms experienced by some patients (so-called long COVID syndrome). The aim of our study was to evaluate the value of a panel of serum biomarkers of severity and prognosis in patients hospitalized for COVID-19 and also as predictive factors for the development of post-COVID lung sequelae after discharge from the hospital.

Krebs von den Lungen-6 (KL-6) in cerebrospinal fluid from neurosarcoidosis patients.

Background: Krebs von den Lungen-6 (KL-6) is a high molecular weight (MW) glycoprotein mainly secreted by type II pneumocytes because of lung damage or during regeneration. Neurosarcoidosis (NS), where sarcoid granulomas involve the nervous system, occurs in 5-20% of patients with sarcoidosis. No data is currently available on KL-6 in serum or CSF of NS patients.

Benralizumab affects NK cell maturation and proliferation in severe asthmatic patients.

Introduction: The mechanism of action of benralizumab is determined by its afucosylated constant fragment that binds CD16a receptors on the membrane of natural killer cells. Here we analysed changes in Natural Killer and T-cells in Severe asthmatic patients, before and after benralizumab.

Methods: Natural Killer and T-cell subsets were detected through multiparametric flow cytometry.

Imbalance of Lymphocyte Subsets and CD45RA-Expressing Cells in Intrathoracic Lymph Nodes, Alveolar Compartment and Bloodstream of Pulmonary Sarcoidosis Patients.

Sarcoidosis is a systemic granulomatous disease mainly affecting the lungs and hilomediastinal lymph nodes. It is characterized by non-caseating epithelioid cell granulomas in lymph nodes and lungs. Our study aimed to evaluate and compare T, B and NK cell subsets in the alveolar compartment, lymph nodes and the bloodstream simultaneously in the same patients to elucidate the immune responses associated with the development and progression of sarcoidosis.