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.

Evaluation of Nailfold Capillaroscopy as a Novel Tool in the Assessment of Eosinophilic Granulomatosis with Polyangiitis.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA), represent a spectrum of systemic disorders characterized by necrotizing inflammation of small- to medium-sized vessels. Nailfold videocapillaroscopy (NVC) is a validated, non-invasive technique routinely employed in the assessment of microvascular involvement in systemic sclerosis and in the differential diagnosis of Raynaud's phenomenon; its application in the context of AAV, particularly EGPA, has not been investigated yet. The present study aims to assess the presence and the possible pattern of microcirculatory abnormalities detected by NVC in EGPA patients, and to explore potential correlations between capillaroscopic findings and disease activity status.

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.

A Simple Ratio in a Complex Disease: Exploring the Neutrophil-to-Lymphocyte Ratio in Idiopathic Pulmonary Fibrosis.

The neutrophil-to-lymphocyte ratio (NLR) is a simple, inexpensive and easily accessible inflammatory biomarker that reflects the balance between innate and adaptive immunity. In recent years, NLR has emerged as a potential prognostic and disease severity marker for different diseases, including idiopathic pulmonary fibrosis (IPF), a progressive and fatal interstitial lung disease with a highly variable course and poor prognosis. Several studies have highlighted that NLR can be associated with several clinical outcomes such as lung function decline, increased risk of hospitalization, acute exacerbation of IPF, and mortality over time.

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.

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.

ICS use trajectories in severe asthma patients on benralizumab: real-life data from 3-years follow-up.

Background: Inhaled steroids dose reduction is a relevant goal in severe asthma management.

Research Question: We aimed to investigate ICS use trajectories and their clinical impact in severe asthma patients on benralizumab over 36 months.

Study Design And Methods: We conducted a retrospective real-life observational study including clinical and inflammatory parameters.

Durability of benralizumab effectiveness in severe eosinophilic asthma patients with and without chronic rhinosinusitis with nasal polyps: a analysis from the ANANKE study.

Introduction: Severe eosinophilic asthma (SEA) often co-occurs with chronic rhinosinusitis with nasal polyps (CRSwNP), worsening asthma symptoms. Earlier studies have shown that benralizumab improves asthma outcomes with greater efficacy if patients present CRSwNP.

Methods: This analysis of the ANANKE study (NCT04272463) reports data on the long-term effectiveness of benralizumab between SEA patients with and without CRSwNP ( = 86 and  = 75, respectively) treated for up to 96 weeks.

Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study.

Background: Following the results of the MANDARA trial, this real-life study aimed at comparing the effectiveness and safety profile of mepolizumab versus benralizumab in a European EGPA cohort.

Methods: We conducted a retrospective observational comparative study including EGPA patients, who received mepolizumab or benralizumab at the asthma dose. Patients were matched 1:1 by sex, age, BVAS and oral corticosteroid (OCS) dosage at the treatment initiation (T0).

The role of heat shock protein 90 in idiopathic pulmonary fibrosis: state of the art.

Heat shock protein 90 (HSP 90) and its isoforms are a group of homodimeric proteins that regulate several cellular processes, such as the elimination of misfolded proteins, cell development and post-translational modifications of kinase proteins and receptors. Due to its involvement in extracellular matrix (ECM) remodelling, myofibroblast differentiation and apoptosis, HSP 90 has been investigated as a key player in the pathogenesis of lung fibrosis. Idiopathic pulmonary fibrosis (IPF) is the most common and deadly interstitial lung disease, due to the progressive distortion of lung parenchyma related to the overproduction and deposition of altered ECM, driven by transforming growth factor-β (TGF-β) dependent and independent pathways.

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.

Dupilumab Effectiveness in Patients with Severe Allergic Asthma Non-Responsive to Omalizumab.

: Severe allergic asthma is usually treated with omalizumab; however, this drug may not be effective for every patient. By its action, dupilumab could be an alternative in these patients. The objective of this study was to evaluate the efficacy of dupilumab in patients with severe allergic asthma, non-responsive to omalizumab, according to the maintenance of their oral corticosteroid (OCS) dose, an exacerbation rate decrease, or poor control of the disease, despite optimized treatment.

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.

Profiling Blood Hypereosinophilia in Patients on Dupilumab Treatment for Respiratory Conditions: A Real-Life Snapshot.

Background: A transient and usually asymptomatic increase in blood eosinophil count (BEC) associated with dupilumab treatment has been described. Predicting factors related to the increase in BEC and the occurrence of symptoms are still poorly investigated.

Objective: To investigate frequency, timing, duration, clinical relevance, and potential predictors of the increase in BEC in a real-life multicenter cohort of patients affected by asthma and/or chronic rhinosinusitis with nasal polyps (CRSwNP) treated with dupilumab.

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.

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.

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.

Sarcoid Nodule or Lung Cancer? A High-Resolution Computed Tomography-Based Retrospective Study of Pulmonary Nodules in Patients with Sarcoidosis.

: The objective of this retrospective study was to compare the characteristics of sarcoid nodules and neoplastic nodules using high-resolution computed tomography (HRCT) in sarcoidosis patients. : This is a single-center retrospective study. From 2010 to 2023, among 685 patients affected by pulmonary sarcoidosis, 23 patients developed pulmonary nodules of a suspicious malignant nature.

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.