scholarly journals Real-life prevalence of progressive fibrosing interstitial lung diseases

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Maureen Gagliardi ◽  
Damienne Vande Berg ◽  
Charles-Edouard Heylen ◽  
Sandra Koenig ◽  
Delphine Hoton ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Vital Capacity ◽  
Interobserver Agreement ◽  
Pulmonary Function Tests ◽  
Real Life ◽  
Interstitial Lung Diseases ◽  
Main Diagnosis ◽  
High Resolution Computed Tomography ◽  
Traction Bronchiectasis

AbstractThe concept of progressive fibrosing interstitial lung disease (PF-ILD) has recently emerged. However, real-life proportion of PF-ILDs outside IPF is still hard to evaluate. Therefore, we sought to estimate the proportion of PF-ILD in our ILD cohort. We also determined the proportion of ILD subtypes within PF-ILD and investigated factors associated with PF-ILDs. Finally, we quantified interobserver agreement between radiologists for the assessment of fibrosis. We reviewed the files of ILD patients discussed in multidisciplinary discussion between January 1st 2017 and December 31st 2019. Clinical data, pulmonary function tests (PFTs) and high-resolution computed tomography (HRCTs) were centrally reviewed. Fibrosis was defined as the presence of traction bronchiectasis, reticulations with/out honeycombing. Progression was defined as a relative forced vital capacity (FVC) decline of ≥ 10% in ≤ 24 months or 5% < FVC decline < 10% and progression of fibrosis on HRCT in ≤ 24 months. 464 consecutive ILD patients were included. 105 had a diagnosis of IPF (23%). Most frequent non-IPF ILD were connective tissue disease (CTD)-associated ILD (22%), hypersensitivity pneumonitis (13%), unclassifiable ILD (10%) and sarcoidosis (8%). Features of fibrosis were common (82% of CTD-ILD, 81% of HP, 95% of uILD). After review of HRCTs and PFTs, 68 patients (19% of non-IPF ILD) had a PF-ILD according to our criteria. Interobserver agreement for fibrosis between radiologists was excellent (Cohen’s kappa 0.86). The main diagnosis among PF-ILD were CTD-ILD (36%), HP (22%) and uILD (20%). PF-ILD patients were significantly older than non-F-ILD (P = 0.0005). PF-ILDs represent about 20% of ILDs outside IPF. This provides an estimation of the proportion of patients who might benefit from antifibrotics. Interobserver agreement between radiologists for the diagnosis of fibrotic ILD is excellent.

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 Automatic quantitative computed tomography measurement of longitudinal lung volume loss in interstitial lung diseases

2022 ◽  
Author(s):  
Salim A. Si-Mohamed ◽  
Mouhamad Nasser ◽  
Marion Colevray ◽  
Olivier Nempont ◽  
Pierre-Jean Lartaud ◽  
...  
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Volume Loss ◽  
Lung Capacity ◽  
Lung Ct ◽  
Ct Volume

Abstract Objectives To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann–Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated. Results In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p < 0.0001). In 408 patients (3332 CT), median annual CTvol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p < 0.0001) over 5.03 years. In 73 IPF patients, a relative annual CTvol loss of 7.9% was associated with major adverse events (log-rank, p < 0.0001) in univariate analysis (p < 0.001). Conclusions Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD. Key Points • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p < 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p < 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p < 0.0001).

scholarly journals Hypersensitivity Pneumonitis: Diagnostic and Therapeutic Challenges

2021 ◽  
Vol 8 ◽  
Author(s):  
Maria Laura Alberti ◽  
Emily Rincon-Alvarez ◽  
Ivette Buendia-Roldan ◽  
Moises Selman
Keyword(s):  
Genetic Risk ◽  
Lung Diseases ◽  
Inflammatory Process ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Lung Diseases ◽  
Therapeutic Options ◽  
High Resolution Computed Tomography ◽  
Multidisciplinary Assessment ◽  
Persistent Inflammation ◽  
Antigen Removal

Hypersensitivity pneumonitis (HP) is one of the most common interstitial lung diseases (ILD), that presents unique challenges for a confident diagnosis and limited therapeutic options. The disease is triggered by exposure to a wide variety of inciting antigens in susceptible individuals which results in T-cell hyperactivation and bronchioloalveolar inflammation. However, the genetic risk and the pathogenic mechanisms remain incompletely elucidated. Revised diagnostic criteria have recently been proposed, recommending to classify the disease in fibrotic and non-fibrotic HP which has strong therapeutic and outcome consequences. Confident diagnosis depends on the presence of clinical features of ILD, identification of the antigen(s), typical images on high-resolution computed tomography (HRCT), characteristic histopathological features, and lymphocytosis in the bronchoalveolar lavage. However, identifying the source of antigen is usually challenging, and HRCT and histopathology are often heterogeneous and not typical, supporting the notion that diagnosis should include a multidisciplinary assessment. Antigen removal and treating the inflammatory process is crucial in the progression of the disease since chronic persistent inflammation seems to be one of the mechanisms leading to lung fibrotic remodeling. Fibrotic HP has a few therapeutic options but evidence of efficacy is still scanty. Deciphering the molecular pathobiology of HP will contribute to open new therapeutic avenues and will provide vital insights in the search for novel diagnostic and prognostic biomarkers.

scholarly journals Utility of serological biomarker’ panels for diagnostic accuracy of interstitial lung diseases

2020 ◽  
Vol 68 (6) ◽  
pp. 414-421
Author(s):  
Laura Bergantini ◽  
Miriana d’Alessandro ◽  
Lucia Vietri ◽  
Giuseppe Domenico Rana ◽  
Paolo Cameli ◽  
...  
Keyword(s):  
Logistic Regression ◽  
Differential Diagnosis ◽  
Diagnostic Accuracy ◽  
Regression Model ◽  
Lung Diseases ◽  
Logistic Regression Model ◽  
Hypersensitivity Pneumonitis ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Reactive Protein

AbstractInterstitial lung diseases (ILD) are a heterogeneous group of illnesses of known and unknown aetiology. Differential diagnosis among the three disorders is often challenging. Specific biomarkers with good sensitivity and specificity are therefore needed to predict clinical outcome and guide clinical decisions. The aim of this study was to investigate inflammatory/fibrotic biomarkers, to determine whether single mediators or panels of mediators could be useful to stratify patients into three distinct domains: sarcoidosis, idiopathic pulmonary fibrosis (IPF) and chronic hypersensitivity pneumonitis (cHP). A total of 163 ILD patients monitored at Siena Referral Centre for Sarcoidosis and other Interstitial Lung Diseases were enrolled in the study. Clinical data, pulmonary function tests and biochemical analytes were retrospectively collected. SAA levels were detected by ELISA kit and Krebs von den Lungen 6 (KL-6) were measured by CLEIA method, for sarcoidosis, cHP and IPF patients. Multiple comparison analysis showed significant differences in C reactive protein (CRP), white blood cell count (WBC) and creatinine levels between the three groups. In the logistic regression model, KL-6, CRP and WBC showed areas under curves (AUC) 0.86, for sarcoidosis diagnosis. The logistic regression model KL-6 and SAA showed the best performance with an AUC 0.81 for discriminating IPF than cHP and sarcoidosis. For differential diagnosis of IPF and cHP, KL-6 and SAA were considered in the logistic regression model, showed an AUC 0.79. The combination of serum biomarkers proposed here offers insights into the pathobiology of ILDs. These panels of bioindicators will improve diagnostic accuracy and will be useful in the clinical management of ILDs.

scholarly journals Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial

2021 ◽  
pp. 2004538
Author(s):  
Kevin R. Flaherty ◽  
Athol U. Wells ◽  
Vincent Cottin ◽  
Anand Devaraj ◽  
Yoshikazu Inoue ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Clinical Practice ◽  
High Resolution ◽  
Lung Diseases ◽  
Vital Capacity ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
Primary Analysis ◽  
High Resolution Computed Tomography ◽  
To Receive

The primary analysis of the INBUILD trial showed that in subjects with progressive fibrosing interstitial lung diseases (ILDs), nintedanib slowed the decline in forced vital capacity (FVC) over 52 weeks. We report the effects of nintedanib on ILD progression over the whole trial.Subjects with fibrosing ILDs other than idiopathic pulmonary fibrosis, who had ILD progression within the 24 months before screening despite management deemed appropriate in clinical practice, were randomised to receive nintedanib or placebo. Subjects continued on blinded randomised treatment until all subjects had completed the trial. Over the whole trial, mean (sd) exposure to trial medication was 15.6 (7.2) and 16.8 (5.8) months in the nintedanib and placebo groups, respectively.In the nintedanib (n=332) and placebo (n=331) groups, respectively, the proportions of subjects who had ILD progression (absolute decline in FVC ≥10% predicted) or died were 40.4% and 54.7% in the overall population (HR 0.66 [95% CI: 0.53, 0.83]; p=0.0003), and 43.7% and 55.8% among subjects with a usual interstitial pneumonia (UIP)-like fibrotic pattern on high-resolution computed tomography (HRCT) (HR 0.69 [0.53, 0.91]; p=0.009). In the nintedanib and placebo groups, respectively, the proportions who had an acute exacerbation of ILD or died were 13.9% and 19.6% in the overall population (HR 0.67 [95% CI: 0.46, 0.98]; p=0.04), and 15.0% and 22.8% among subjects with a UIP-like fibrotic pattern on HRCT (HR 0.62 [0.39, 0.97]; p=0.03).Based on data from the whole INBUILD trial, nintedanib reduced the risk of events indicating ILD progression.

scholarly journals Monitoring and management of fibrosing interstitial lung diseases: a narrative review for practicing clinicians

2021 ◽  
Vol 15 ◽  
pp. 175346662110397
Author(s):  
Anoop M. Nambiar ◽  
Christopher M. Walker ◽  
Jeffrey A. Sparks
Keyword(s):  
Lung Diseases ◽  
Multidisciplinary Approach ◽  
Progressive Disease ◽  
Pulmonary Function Tests ◽  
Interstitial Lung Diseases ◽  
Narrative Review ◽  
Disease Monitoring ◽  
Close Monitoring ◽  
High Resolution Computed Tomography ◽  
Progression Risk

Close monitoring of patients with fibrosing interstitial lung diseases (ILDs) is important to enable prompt identification and management of progressive disease. Monitoring should involve regular assessment of physiology (including pulmonary function tests), symptoms, and, when appropriate, high-resolution computed tomography. The management of patients with fibrosing ILDs requires a multidisciplinary approach and should be individualized based on factors such as disease severity, evidence of progression, risk factors for progression, comorbidities, and the preferences of the patient. In this narrative review, we discuss how patients with fibrosing ILDs can be effectively monitored and managed in clinical practice.

scholarly journals The Role of Transthoracic Ultrasound in the Study of Interstitial Lung Diseases: High-Resolution Computed Tomography Versus Ultrasound Patterns: Our Preliminary Experience

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 439
Author(s):  
Donato Lacedonia ◽  
Giulia Scioscia ◽  
Angelamaria Giardinelli ◽  
Carla Maria Irene Quarato ◽  
Ennio Vincenzo Sassani ◽  
...  
Keyword(s):  
Computed Tomography ◽  
High Resolution ◽  
Lung Diseases ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Pleural Line ◽  
Complementary Role ◽  
Number Of Patients ◽  
Significant Difference ◽  
Transthoracic Ultrasound

Transthoracic ultrasound (TUS) is a readily available imaging tool that can provide a quick real-time evaluation. The aim of this preliminary study was to establish a complementary role for this imaging method in the approach of interstitial lung diseases (ILDs). TUS examination was performed in 43 consecutive patients with pulmonary fibrosis and TUS findings were compared with the corresponding high-resolution computed tomography (HRCT) scans. All patients showed a thickened hyperechoic pleural line, despite no difference between dominant HRCT patterns (ground glass, honeycombing, mixed pattern) being recorded (p > 0.05). However, pleural lines’ thickening showed a significant difference between different HRCT degree of fibrosis (p < 0.001) and a negative correlation with functional parameters. The presence of >3 B-lines and subpleural nodules was also assessed in a large number of patients, although they did not demonstrate any particular association with a specific HRCT finding or fibrotic degree. Results allow us to suggest a complementary role for TUS in facilitating an early diagnosis of ILD or helping to detect a possible disease progression or eventual complications during routine clinical practice (with pleural line measurements and subpleural nodules), although HRCT remains the gold standard in the definition of ILD pattern, disease extent and follow-up.

O40-1 Risk of hypersensitivity pneumonitis and other interstitial lung diseases among pigeon breeders

2016 ◽  
Author(s):  
Christine Cramer ◽  
Vivi Schlünssen ◽  
Elisabeth Bendstrup ◽  
Zara Ann Stokholm ◽  
Jesper Medom Vestergaard ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Interstitial Lung Diseases

scholarly journals Computed tomography findings as determinants of pulmonary function tests in fibrotic interstitial lung diseases—Network-analyses and multivariate models

2020 ◽  
Vol 17 ◽  
pp. 147997312096702
Author(s):  
David Lang ◽  
Kaveh Akbari ◽  
Stefan Walcherberger ◽  
Benedikt Hergan ◽  
Andreas Horner ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Information Criterion ◽  
Forced Expiratory Volume ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Diffusion Capacity ◽  
Network Analyses ◽  
The Impact

The aim was to evaluate the impact of multiple high-resolution computed tomography (HRCT) features on pulmonary function test (PFT) biomarkers in fibrotic interstitial lung disease (FILD) patients. HRCT of subsequently ILD-board-discussed FILD patients were semi-quantitatively evaluated in a standardized approach: 18 distinct lung regions were scored for noduli, reticulation, honeycombing, consolidations, ground glass opacities (GGO), traction bronchiectasis (BRK) and emphysema. Total lung capacity (TLC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/FVC, diffusion capacity for carbon monoxide (DLCO) and transfer coefficient (KCO) were assessed. Interactions between each PFT biomarker and all HRCT scores were visualized by network analyses, modeled according to the Schwarz Bayesian Information Criterion and incorporated in uni- and multivariate stepwise regression analyses. Among 108 FILD patients (mean age 67 years, 77% male), BRK extent was a major significant uni- or multivariate determinant of all PFT analyzed. Besides that, diffusion-based variables DLCO and KCO showed a larger dependency on reticulation, emphysema and GGO, while forced expiratory volume-based measures FEV1, FVC and FEV1/FVC were more closely associated with consolidations. For TLC, the only significant multivariate determinant was reticulation. In conclusion, PFT biomarkers derived from spirometry, body plethysmography and diffusion capacity in FILD patients are differentially influenced by semi-quantified HRCT findings.

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