Role of lung biopsies in the diagnosis of interstitial lung diseases in a clinical real-life setting

2021 ◽  
Author(s):  
Melanie Berger ◽  
Maximilian Wollsching-Strobel ◽  
Elisabeth Bünemann ◽  
Sarah Bettina Schwarz ◽  
Daniel Sebastian Majorski ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Real Life ◽  
Interstitial Lung Diseases ◽  
Real Life Setting ◽  
Lung Biopsies

Progressive Fibrosing Interstitial Lung Diseases: Prevalence and Characterization in Two Italian Referral Centers

Respiration ◽  
2020 ◽  
Vol 99 (10) ◽  
pp. 838-845
Author(s):  
Paola Faverio ◽  
Martina Piluso ◽  
Federica De Giacomi ◽  
Matteo Della Zoppa ◽  
Roberto Cassandro ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Vital Capacity ◽  
Progressive Disease ◽  
Real Life ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Real Life Setting ◽  
History Of

<b><i>Background:</i></b> The prevalence and natural history of progressive fibrosing interstitial lung diseases (PF-ILDs), and their response to commonly used treatments in real life are largely unknown. <b><i>Objectives:</i></b> The aim of the study was to describe the prevalence, clinical characteristics, management, and outcomes of PF-ILD patients attending 2 Italian referral centers (San Gerardo Hospital, Monza, and San Giuseppe Hospital, Milan) from January 1, 2011, to July 31, 2019. <b><i>Methods:</i></b> From a cohort of non-idiopathic pulmonary fibrosis fibrosing ILD patients with at least 2-year follow-up, we selected only those with progressive disease, defined as per the INBUILD trial, collecting their demographical, clinical, and functional data. <b><i>Results:</i></b> Out of the 245 fibrosing ILD patients, 75 (31%) were classified as PF-ILDs (median age 66 years, 60% males), most frequently idiopathic non-specific interstitial pneumonia (28%), followed by connective tissue disease-associated ILD (20%), chronic hypersensitivity pneumonitis, and sarcoidosis (17% each). Most patients (81%) were categorized as PF-ILDs because of forced vital capacity (FVC) decline ≥10%, while 19% experienced a marginal FVC decline (between 5 and 10%) associated with worsening respiratory symptoms or increasing extent of fibrotic changes on high-resolution computed tomography. Disease progression occurred after a median of 18 months from ILD diagnosis. The vast majority (93%) of PF-ILD patients received prednisolone, alone (40%) or associated with steroid-sparing agents (52%), and 35% of treated patients developed treatment-related adverse events. After ILD progression, the median survival was 3 (interquartile range (IQR) 2–5) years, with a 2- and 3-year mortality rate of 4 and 20%, respectively. <b><i>Conclusions:</i></b> In a real-life setting, approximately one-third of the fibrosing ILD patients showed a progressive course despite treatment. Studies aimed to better phenotype this subgroup of patients are needed.

scholarly journals Reply to Wand et al.: Role of Transbronchial Cryobiopsy in Interstitial Lung Diseases: An Ongoing Tale

2020 ◽  
Vol 201 (2) ◽  
pp. 260-261
Author(s):  
Arnaud Bourdin ◽  
Carey M. Suehs ◽  
Thomas V. Colby ◽  
Isabelle Vachier ◽  
Nicolas Molinari ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Interstitial Lung Diseases

The Role of Induced Sputum in Amiodarone-Associated Interstitial Lung Diseases

Cardiology ◽  
2006 ◽  
Vol 108 (4) ◽  
pp. 223-227 ◽  
Author(s):  
Elizabeth Fireman ◽  
Ian Topilsky ◽  
Sami Viskin ◽  
Israel E. Priel
Keyword(s):  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Induced Sputum

scholarly journals The Impact of Multidisciplinary Discussion (MDD) in the Diagnosis and Management of Fibrotic Interstitial Lung Diseases

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
Ghofran Ageely ◽  
Carolina Souza ◽  
Kaissa De Boer ◽  
Saly Zahra ◽  
Marcio Gomes ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Significant Proportion ◽  
Real Life ◽  
Interstitial Lung Diseases ◽  
Limited Information ◽  
Reference Standard ◽  
Provisional Diagnosis ◽  
Surgical Biopsy ◽  
Academic Center ◽  
The Impact

Accurate diagnosis of interstitial lung disease (ILD) is crucial for management and prognosis but can be challenging even for experienced clinicians. Expert multidisciplinary discussion (MDD) is considered the reference standard for ILD diagnosis; however, there remain concerns regarding lack of validation studies and relative limited information on the impact of MDD in real-life clinical practice. The goal of this study was to assess the effect of MDD in providing a specific ILD diagnosis, changing the diagnosis provided upon referral, and to determine how often and in which way MDD altered management. Material and Methods. Retrospective observational study in an ILD referral tertiary academic center. MDD diagnoses were categorized as specific, provisional, and unclassifiable ILD. Pre-MDD and MDD diagnoses were compared for change in diagnosis and concordance rates for specific diagnoses. Relevant change in management including initiation or change in pharmacological treatment, referral to surgical biopsy, and nonpharmacological management were recorded. Results. 126 cases were included (79M, 47F, 36–93 years, mean 70 y). Specific MDD diagnosis was provided in 62% (78/126); 12% (15/126) had provisional diagnosis, and 21% (27/126) was unclassifiable. Overall agreement for specific pre-MDD and MDD diagnosis was 41% (52/126) and 80% for idiopathic pulmonary fibrosis (IPF) diagnosis. MDD altered diagnosis in 37% (47/126) and changed management in 39% (50/126). Amongst concordant diagnoses, management was altered in 46% (24/52). In summary, MDD provided a specific diagnosis discordant with pre-MDD diagnosis in a significant proportion of cases and was particularly valuable in the diagnosis of non-IPF ILD. MDD often altered management and had relevant impact on management even in cases with concordant pre-MDD diagnosis.

scholarly journals Extended Exhaled Nitric Oxide Analysis in Interstitial Lung Diseases: A Systematic Review

2020 ◽  
Vol 21 (17) ◽  
pp. 6187
Author(s):  
Paolo Cameli ◽  
Elena Bargagli ◽  
Laura Bergantini ◽  
Miriana d’Alessandro ◽  
Maria Pieroni ◽  
...  
Keyword(s):  
Nitric Oxide ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Exhaled Nitric Oxide ◽  
Inhalation Therapy ◽  
Asthmatic Patients ◽  
Promising Tool ◽  
Multiple Flows ◽  
Published Evidence

Fractional exhaled nitric oxide (FeNO) is a well-known and widely accepted biomarker of airways inflammation that can be useful in the therapeutic management, and adherence to inhalation therapy control, in asthmatic patients. However, the multiple-flows assessment of FeNO can provide a reliable measurement of bronchial and alveolar production of NO, supporting its potential value as biomarker also in peripheral lung diseases, such as interstitial lung diseases (ILD). In this review, we first discuss the role of NO in the pathobiology of lung fibrosis and the technique currently approved for the measurement of maximum bronchial flux of NO (J’awNO) and alveolar concentration of NO (CaNO). We systematically report the published evidence regarding extended FeNO analysis in the management of patients with different ILDs, focusing on its potential role in differential diagnosis, prognostic evaluation and severity assessment of disease. The few available data concerning extended FeNO analysis, and the most common comorbidities of ILD, are explored too. In conclusion, multiple-flows FeNO analysis, and CaNO in particular, appears to be a promising tool to be implemented in the diagnostic and prognostic pathways of patients affected with ILDs.

scholarly journals A single-institution study of concordance of pathological diagnoses for interstitial lung diseases between pre-transplantation surgical lung biopsies and lung explants

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Tanmay S. Panchabhai ◽  
Andrea Valeria Arrossi ◽  
Kristin B. Highland ◽  
Debabrata Bandyopadhyay ◽  
Daniel A. Culver ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
Single Institution ◽  
Lung Biopsies
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