Publications by authors named "Luca Richeldi"

Exposure to air pollution has been associated with up to 9 million premature deaths per year worldwide, with the respiratory system a key site for its effects. Air pollution exposure is a well-established risk factor for the development and exacerbation of airways diseases and lung cancer, however relatively little is known regarding the risks associated with air pollution interacting with areas of gas exchange - the alveoli and pulmonary interstitium. In recent years, evidence has emerged identifying a role in the development and progression of sub-clinical interstitial lung abnormalities as well as progression and risk of exacerbation of fibrotic interstitial lung diseases.

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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.

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Background: Despite advances in diagnosis and management, the interstitial lung disease (ILD) lexicon is plagued by ambiguous and inconsistent terminology that complicates communication and impedes knowledge generation. The objective of this Fleischner Society Consensus Statement was to produce standardized terminology for ILD multidisciplinary diagnoses and major phenotypes.

Methods: Interviews with 10 experts were used to identify ILD clinical diagnoses and major phenotypes.

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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.

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Background: Thoracoscopy guidelines recommend inducing artificial pneumothorax before medical thoracoscopy in patients with minimal or absent pleural effusion. Recent single-arm studies have demonstrated that non-artificial pneumothorax approaches reduce operative time and complication rates compared with artificial pneumothorax techniques in these patients. However, there is a lack of trials comparing the effectiveness and safety of performing artificial pneumothorax versus not performing it in these cases.

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Residual lung abnormalities on CT scans after COVID-19 respiratory infection may be associated with persistent or progressive respiratory symptoms and frequently correlate with abnormal pulmonary function testing results. These abnormalities have been described using varying terms in numerous publications. Chest CT lung abnormalities after COVID-19 infection tend to stabilize or regress over time, indicating that they are nonprogressive and postinfectious in nature.

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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.

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Background: Nerandomilast (BI 1015550) is an orally administered preferential inhibitor of phosphodiesterase 4B with antifibrotic and immunomodulatory properties. Nerandomilast has been shown to slow the progression of idiopathic pulmonary fibrosis, but an assessment of its effects in other types of progressive pulmonary fibrosis is needed.

Methods: In a phase 3, double-blind trial, we randomly assigned patients with progressive pulmonary fibrosis in a 1:1:1 ratio to receive nerandomilast at a dose of 18 mg twice daily, nerandomilast at a dose of 9 mg twice daily, or placebo, with stratification according to background therapy (nintedanib vs.

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There is growing interest in identifying early stages of interstitial lung disease (ILD) to improve patient outcomes. This document reviews updated evidence on interstitial lung abnormalities (ILAs); provides suggestions for screening, evaluation, and management; proposes criteria for distinguishing ILAs from ILD; and identifies research priorities. A committee of clinical and methodology experts met by video conference to define ILAs and ILD by consensus and voted on 11 prespecified questions after reviewing synthesized evidence from a systematic literature search.

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Background: Nerandomilast (BI 1015550) is an orally administered preferential inhibitor of phosphodiesterase 4B with antifibrotic and immunomodulatory effects. In a phase 2 trial involving patients with idiopathic pulmonary fibrosis, treatment with nerandomilast stabilized lung function over a period of 12 weeks.

Methods: In this phase 3, double-blind trial, we randomly assigned patients with idiopathic pulmonary fibrosis in a 1:1:1 ratio to receive nerandomilast at a dose of 18 mg twice daily, nerandomilast at a dose of 9 mg twice daily, or placebo, with stratification according to background antifibrotic therapy (nintedanib or pirfenidone vs.

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Mepolizumab represents an effective strategy for severe eosinophilic asthma. Small airways disease (SAD) defines a peculiar asthma phenotype related to worse disease control. Limited and indirect findings are currently available on the effect of mepolizumab on SAD.

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Background: Interstitial lung diseases (ILDs) are a heterogeneous group of conditions that can cause fibrosis of the lung interstitium, resulting in respiratory failure and death. Patients with an ILD, particularly idiopathic pulmonary fibrosis (IPF) or connective tissue disease-associated ILDs (CTD-ILDs), are prone to develop chronic pulmonary infections such as tuberculosis (TB) and non-tuberculous mycobacterial pulmonary disease (NTM-PD).

Methods: This case series examines the management of three ILD patients with a usual interstitial pneumonia (UIP) pattern and concomitant NTM-PD or TB at National Institute for Infectious Diseases "Lazzaro Spallanzani" in Rome, Italy, over three years (2019-2022).

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As COVID-19 has become an epidemic, we conducted an open-label study aimed to identify immunogenicity and reactogenicity of boosters of the BNT162b2 vaccine in a real-world cohort of long-survivor metastatic lung cancer patients (LS-mLC pts). According to the timing of the booster dose (BD) and SARS-CoV-2 infection (Cov-I) during anticancer treatment (ACT), between October 2021 and February 2022, we prospectively enrolled 166 cancer patients into five parallel cohorts. The primary endpoints were seroprevalence of IgG Anti-spike-RBD (anti-S IgG) at two pre-defined timepoints (T1: +30-90 days after BD; T2: +6 months +/- 4 weeks after BD).

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Background: Idiopathic pulmonary fibrosis (IPF) and progressive pulmonary fibrosis (PPF) are chronic fibrosing interstitial lung diseases associated with irreversible loss of lung function and early mortality. Admilparant (BMS-986278) is an oral lysophosphatidic acid receptor 1 antagonist under development for treatment of IPF and PPF.

Research Question: How does admilparant affect time to disease progression in patients with IPF or PPF?

Study Design And Methods: In a phase 2, randomized, double-anonymized, placebo-controlled study, parallel cohorts of patients with IPF or PPF were randomized separately 1:1:1 to receive 30 mg admilparant, 60 mg admilparant, or placebo twice daily for 26 weeks; background antifibrotics were allowed.

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New ultrathin bronchoscopes (UTBs) enable the inspection and biopsy of small airways, potentially offering diagnostic advantages in sarcoidosis. In this prospective study, patients with suspected sarcoidosis underwent airway inspection with a UTB. Observed airway abnormalities were categorised into six predefined patterns.

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Patients with pulmonary fibrosis (PF) often experience long waits before getting a correct diagnosis, and this delay in reaching specialized care is associated with increased mortality, regardless of the severity of the disease. Early diagnosis and timely treatment of PF can potentially extend life expectancy and maintain a better quality of life. Crackles present in the recorded lung sounds may be crucial for the early diagnosis of PF.

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Patients with pulmonary fibrosis (PF) often experience long waits before getting a correct diagnosis, and this delay in reaching specialized care is associated with increased mortality, regardless of the severity of the disease. Early diagnosis and timely treatment of PF can potentially extend life expectancy and maintain a better quality of life. Crackles present in the recorded lung sounds may be crucial for the early diagnosis of PF.

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Comprehensive care integrates individual patient needs and is highly valued for patients with pulmonary fibrosis (PF). The importance of a patient-centered care approach is rooted in the unpredictable progressiveness of the disease course in PF. The respiratory impairment associated with PF has a major impact on the quality of life for both patients and their caregivers.

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Background: Although crackles on chest auscultation represent a fundamental component of the diagnostic suspect for fibrotic interstitial lung disease (ILD), their reliability has not been properly studied. We assessed the agreement among respiratory physicians on the presence and changes over time of audible crackles collected in a prospective longitudinal cohort of patients with fibrotic ILD.

Methods: Lung sounds were digitally recorded at baseline and after 12 months at eight anatomical sites.

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Article Synopsis
  • Familial Pulmonary Fibrosis (FPF) is a lung disease caused by genetic mutations, and managing it is still a challenge as of 2023.
  • A survey conducted in Italy found that more FPF patients are seen at hospitals that treat many interstitial lung disease (ILD) patients each year.
  • There were differences in genetic testing services between hospitals, but no major differences in patient care or treatments were found in various medical settings.
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Matrix stiffening by lysyl oxidase-like 2 (LOXL2)-mediated collagen cross-linking is proposed as a core feedforward mechanism that promotes fibrogenesis. Failure in clinical trials of simtuzumab (the humanized version of AB0023, a monoclonal antibody against human LOXL2) suggested that targeting LOXL2 may not have disease relevance; however, target engagement was not directly evaluated. We compare the spatial transcriptome of active human lung fibrogenesis sites with different human cell culture models to identify a disease-relevant model.

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Interstitial lung disease (ILD) in systemic sclerosis (SSc) is a common complication that has a varied progression rate and prognosis. Different progression definitions are available, including minimal clinically important worsening of FVC, EUSTAR (European Scleroderma Trials and Research Group) progression, OMERACT (Outcome Measures in Rheumatology Clinical Trials) progression, and Erice ILD working group progression. Pulmonary function and symptom changes may act as specific confounding factors when applying these definitions in SSc.

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Article Synopsis
  • * An adult woman with ongoing respiratory issues underwent extensive genetic testing, leading to the identification of the novel haplotype F508del/E583G, with bioinformatics analyses suggesting that the E583G variant may be harmful.
  • * The research highlights the importance of updated CFTR-RD guidelines, emphasizing the need for thorough evaluations when discovering new variants to provide better diagnostic and treatment options.
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