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 Morphological Patterns of Sarcoidosis and Clinical Outcome: Retrospective Analysis through a Multidisciplinary Approach

Diagnostics ◽  
2020 ◽  
Vol 10 (4) ◽  
pp. 212 ◽  
Author(s):  
Giulio Distefano ◽  
Ada Vancheri ◽  
Monica Palermo ◽  
Francesco Tiralongo ◽  
Pietro Valerio Foti ◽  
...  
Keyword(s):  
Multidisciplinary Approach ◽  
Pulmonary Function Tests ◽  
Inverse Correlation ◽  
Clinical History ◽  
Interstitial Lung Diseases ◽  
University Hospital ◽  
High Resolution Computed Tomography ◽  
Atypical Manifestations ◽  
Hrct Patterns ◽  
Morphological Patterns

The aim of this work was to verify the correlations between different pulmonary morphological patterns and functional outcomes in sarcoidosis patients, using a validated score for the comparison between the high-resolution computed tomography (HRCT) of patients belonging to different imaging patterns. From the electronic database of the reference center for interstitial lung diseases of our University Hospital, we retrospectively selected 55 patients with a diagnosis of sarcoidosis according to the American Thoracic Society (ATS) criteria; we evaluated the initial HRCT examination and pulmonary function tests collected at baseline and after a year. Patients were divided into typical (48% of patients) and atypical (52%) HRCT patterns, and a computer tomography activity score (CTAS) was associated with each HRCT appearance detected; clinical history, impact of therapy, and extra-thoracic locations were also considered. We found that worsening of diffusing capacity for carbon monoxide (DLCO) is related to the CTAS (r = −0.20, p = 0.01), and there was an inverse correlation between the variation of forced vital capacity (FVC) and the value of the CTAS (r = −0.30, p = 0.23) in the subgroup of patients with atypical patterns. CTAS were higher in patients with extra-pulmonary localizations (p = 0.05) and the subgroup of patients with extra-thoracic locations and atypical manifestations had a greater worsening in terms of variation of FVC (p = 0.03) and DLCO% (p = 0.04). No difference between treated and untreated patients was found.

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.

scholarly journals State of the art in the diagnosis and management of interstitial lung disease

2015 ◽  
Vol 88 (2) ◽  
pp. 116-123 ◽  
Author(s):  
Maria T. A. Buzan ◽  
Carmen Monica Pop
Keyword(s):  
Multidisciplinary Approach ◽  
Pulmonary Function Tests ◽  
Blood Gas Analysis ◽  
Final Diagnosis ◽  
Interstitial Lung Diseases ◽  
Gas Analysis ◽  
High Resolution Computed Tomography ◽  
Pulmonary Parenchyma ◽  
6 Minute Walk Test ◽  
Minute Walk

The interstitial lung diseases (ILDs) are a diverse group of disorders characterized by a varying combination of inflammation and fibrosis of the pulmonary parenchyma. Treatment and prognosis of ILD typically depend on the underlying ILD subtype, highlighting the importance of accurate classification and diagnosis. Besides a thorough history and clinical examination, the protocol should include a 6-minute walk test, chest radiography, high-resolution computed tomography, biochemical analysis, pulmonary function tests, blood gas analysis, bronchoalveolar lavage, and, when necessary, a lung biopsy. The final diagnosis of ILD entities requires dynamic interaction between clinicians, radiologists and pathologists to reach a clinico-radiologic-pathologic diagnosis, the gold standard no longer being the histology but rather a multidisciplinary approach.

scholarly journals Idiopathic pulmonary fibrosis: possibilities of multidisciplinary diagnostic approach

2018 ◽  
Vol 28 (5) ◽  
pp. 622-625
Author(s):  
A. М. Kardangusheva ◽  
Н. A. Sabanchieva
Keyword(s):  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Lung Biopsy ◽  
Poor Prognosis ◽  
Lung Diseases ◽  
Multidisciplinary Approach ◽  
Usual Interstitial Pneumonia ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Antifibrotic Drugs

Idiopathic pulmonary fibrosis (IPF) is the commonest form of idiopathic interstitial pneumonias with very poor prognosis. Currently, diagnostic and treatment approaches to this disease have been revised. Confirmation of the diagnosis requires careful exclusion of other known causes of interstitial lung diseases and the presence of usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRTC) and/or on lung biopsy. Also, multidisciplinary discussion involving experts with experience in the diagnosis of interstitial lung diseases is recommended. Given recent knowledge on pathogenesis of IPF antifibrotic drugs are recommended for the therapy of this disease. A clinical case that demonstrates the multidisciplinary approach to diagnosis of IPF is reported in this article.

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 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.

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.

scholarly journals Factors associated with heart failure in patients with interstitial lung diseases

2018 ◽  
Vol 96 (9) ◽  
pp. 814-819
Author(s):  
Elena I. Leonova ◽  
E. I. Shmelev ◽  
I. E. Stepanyan ◽  
E. F. Shergina ◽  
A. S. Zaitseva
Keyword(s):  
Heart Failure ◽  
Right Ventricle ◽  
Lung Diseases ◽  
Target Therapy ◽  
Systolic Dysfunction ◽  
Interstitial Lung Diseases ◽  
High Resolution Computed Tomography ◽  
Basal Diameter ◽  
Factors Associated ◽  
Lung Capacity

Introduction. Investigation of relationships between interstitial lung diseases (ILD) and development of heart failure would be useful for determining target therapy of heart failure (HF) among such patients. Aims.To study intracardiac hemodynamic and factors associated with its dysfunction among patients with ILD. 40 patients including: 34 with Chronic Hypersensitivity Pneumonitis (HP) and 6 with idiopathic pulmonary fibrosis (IPF). Methods. Pulmonary high-resolution computed tomography, echocardiography, spirometry, diffusing lung capacity for carbon monoxide (DLCO), bodypletysmography, blood oxygen (PaO ) were estimated. Results. 55.9% of patients with HP and all patients with IPF had a «cor pulmonale» with diastolic disfunction (DD), and 40% of them also had systolic dysfunction of right ventricle (RV). DD of left ventricle (LV) of I type was found among 10% of patients. 20% of patients were diagnosed with high pulmonary pressure (PP). The basal diameter of right ventricle (BDRV) correlated with DLCO (r=-0,718, p=0,004), PaO (r=-0,690, p=0,006), lung fibrosis (LF) (r=0,602, p=0,002). Tricuspid Annular Plane Systolic Excursion (TAPSE) correlated with mitral E/E’ ratio, tricuspid E/E’ ratio, total lung capacity (TLC) and LF (r=-0,753, p=0,002). Parameters of DD of RV significantly correlated with BDRV, TLC, DLCO, LF. Mitral E/E’ ratio correlated with DLCO and LF. PP corresponded with DLCO, DD of LV, LF (r=0,536, p=0,03) Conclusions. Correlation of LF with parameters of heart failure makes it one of the targets for ILD therapy as well as HF associated with ILD.

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