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 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 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 Risk of hypersensitivity pneumonitis and interstitial lung diseases among pigeon breeders

2016 ◽  
Vol 48 (3) ◽  
pp. 818-825 ◽  
Author(s):  
Christine Cramer ◽  
Vivi Schlünssen ◽  
Elisabeth Bendstrup ◽  
Zara Ann Stokholm ◽  
Jesper Medom Vestergaard ◽  
...  
Keyword(s):  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Cox Regression ◽  
Interstitial Lung Diseases ◽  
Protective Measures ◽  
National Patient Registry ◽  
Hazard Ratios ◽  
Increased Risk ◽  
National Patient

We studied the risk of hypersensitivity pneumonitis and other interstitial lung diseases (ILDs) among pigeon breeders.This is a retrospective follow-up study from 1980 to 2013 of 6920 pigeon breeders identified in the records of the Danish Racing Pigeon Association. They were compared with 276 800 individually matched referents randomly drawn from the Danish population. Hospital based diagnoses of hypersensitivity pneumonitis and other ILDs were identified in the National Patient Registry 1977–2013. Stratified Cox regression analyses estimated the hazard ratios (HR) of hypersensitivity pneumonitis and other ILDs adjusted for occupation, residence and redeemed prescription of medication with ILDs as a possible side-effect. Subjects were censored at death, emigration or a diagnosis of connective tissue disease.The overall incidence rate of ILD was 77.4 per 100 000 person-years among the pigeon breeders and 50.0 among the referents. This difference corresponded to an adjusted HR of 1.56 (95% CI 1.26–1.94). The adjusted HRs of hypersensitivity pneumonitis and other ILDs for pigeon breeders were 14.36 (95% CI 8.10–25.44) and 1.33 (95% CI 1.05–1.69), respectively.This study shows an increased risk of ILD among pigeon breeders compared with the referent population. Protective measures are recommended even though ILD leading to hospital contact remains rare among pigeon breeders.

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.

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

scholarly journals POS0693 EFFICACY AND SAFETY OF BELIMUMAB IN PATIENTS WITH LUPUS NEPHRITIS IN REAL-LIFE SETTING: RESULTS FROM A LARGE, NATIONWIDE, MULTICENTRIC, PROSPECTIVE COHORT

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 594-595
Author(s):  
F. Saccon ◽  
M. Gatto ◽  
M. Zen ◽  
M. Fredi ◽  
F. Regola ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Independent Predictor ◽  
Rescue Therapy ◽  
Real Life ◽  
Multivariate Logistic Regression Analysis ◽  
Class Iii ◽  
Baseline Serum ◽  
Renal Response ◽  
Real Life Setting

Background:LN is still a severe manifestation of Systemic lupus erythematosus (SLE) and multitarget therapy is needed to control the disease especially in refractory cases.Objectives:To evaluate renal response in SLE patients with glomerulonephritis (GN) treated with Belimumab in real-life setting.Methods:Patients with proteinuria >0.5 g/24 h and/or active sediment at baseline enrolled in a multicentre Italian cohort of SLE patients (BeRLiSS study), treated with monthly iv Belimumab 10 mg/kg plus standard of care were considered in this study. Complete renal response (CRR) was defined as proteinuria <0.5 g/24 h, estimated glomerular filtration rate (eGFR)≥90ml/min/1.73m2 and no rescue therapy. Primary efficacy renal response (PERR) was defined as proteinuria ≤0.7 g/24 h, eGFR ≥60ml/min/1.73m2 and no rescue therapy. Prevalence and predictive factors of CRR and PERR at 12 and 24 months after Belimumab initiation were analyzed by multivariate logistic regression analysis.Results:A total of 91 patients were considered in this study, 79 female, mean age 40.51±9.03 years, mean disease duration 12.18±8.15 years, median follow-up time after Belimumab initiation 22 months. Twenty patients had baseline proteinuria ≥0.5 <1 g/day, 17 ≥1 <2 g/day, 13 ≥2 g/day. Belimumab was started at GN onset in 20 (22%) patients and at the time of a renal flare in all other cases. Seventy-five patients underwent a renal biopsy: 1 class I, 4 class II, 14 class III, 47 class IV and 9 class V. Baseline serum creatinine was 82.44±29.26 umol/L; 15 patients showed eGFR<60ml/min/1.73m2 at baseline. Immunosuppresants were taken by 70 (76.9%) patients: 47 micofenolate, 15 azathioprine and 5 ciclosporine. Sixty patients (65.9%) were on antimalarials. During follow-up 34 (37.4%) patients achieved CRR. Among them 5 (14.7%) patients relapsed and 29 (85.3%) patients maintained remission. Mean time to achieved CRR was 9.71±5.91 months.High levels of baseline proteinuria were a negative independent predictor of CRR and PERR at 6 months (OR 0.044 CI95% 0.006-0.320 p=0.002 and OR 0.232 CI95% 0.091-0.596 p=0.002) and 12 months (OR 0.029 CI95% 0.002-0.556 p=0.019 and OR 0.056 CI95% 0.009-0.327 p=0.001). High levels of baseline creatinine were a negative independent predictor of renal response. Renal response at 6 months was a strong predictive factor of renal response at 12 and 24 months.Conclusion:Belimumab is an effective add-on therapy in the treatment of GN in real-life practice setting.Disclosure of Interests:None declared

scholarly journals FRI0495 FOLLOW UP OF INTERSTITIAL PNEUMONIA WITH AUTOIMMUNE FEATURES – THE EXPERIENCE OF ONE CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 846.3-846
Author(s):  
N. Madeira ◽  
M. Alvarenga Santos ◽  
L. Cunha Miranda ◽  
S. Clemente ◽  
S. Furtado
Keyword(s):  
Interstitial Pneumonia ◽  
Respiratory Symptoms ◽  
Interstitial Lung Diseases ◽  
European Respiratory Society ◽  
High Resolution Computed Tomography ◽  
Disease Evolution ◽  
Clinical Criteria ◽  
Clinical Evolution ◽  
Relative Decline

Background:Interstitial Lung Diseases (ILD) may present features suggesting an underlying autoimmune process, which seem to differentiate them from idiopathic interstitial pneumonias, although without fully meeting the classification criteria (CC) for a specific connective tissue disease. Different terms had been used to describe these conditions and, to reach a consensus, the European Respiratory Society/American Thoracic Society proposed the CC for an entity named Interstitial Pneumonia with Autoimmune Features (IPAF). Clinical evolution and prognosis of this entity are still poorly understood.Objectives:To evaluate clinical evolution and prognosis of a population of patients with IPAF.Methods:Retrospective analysis of clinical files of patients followed by the Pulmonology Department since 02/2012 until 06/2019, who met the CC for IPAF, regarding clinical, functional and radiological evolution. Patients were considered to have a progressive phenotype in 24±3 months from their 1stevaluation if they fulfil 1 of the 4 criteria: relative decline in FVC ≥10% predicted; relative decline in FVC ≥5–<10% predicted and worsened respiratory symptoms; relative decline in FVC ≥5–<10% predicted and increased extent of fibrosis on High-resolution Computed Tomography (HRCT); worsened respiratory symptoms and increased extent of fibrosis on HRCT.Results:22 (7.4%) of 296 ILD patients met IPAF CC. 59.0% were female with an age at the 1stevaluation of 66.7±12.4 years. They were all non-smokers (63.6%) or ex-smokers (36.4%). Serologic and morphologic criteria were both present in 21 (95.4%) and clinical criteria in 5 patients (22.7%). Antinuclear antibodies (ANA) were identified in 19, rheumatoid factor in 4, SSA in 3 and anti-Jo-1 in 1 patient. HRCT patterns were identified in 21 patients: 15 nonspecific interstitial pneumonia (NSIP), 5 organizing pneumonia (OP) and 2 lymphocytic interstitial pneumonia (LIP). One NSIP and 1 LIP identified on HRCT were confirmed by histopathology. Three patients had inflammatory arthritis and 2 had Raynaud’s phenomenon. Immunosuppressive therapy was introduced in most cases (18 patients, including systemic corticotherapy in 17, azathioprine in 4, mycophenolate mofetil in 1), azithromycin was prescribed in 2 patients and 3 remained without therapy. Regarding the follow up at 24±3 months from the 1stevaluation (3 patients were excluded due to too recent follow-up), 4 patients (18.2%) had progressive phenotype, 7 (31.8%) had a favourable evolution and 3 (13.6%) patients had died. During a follow-up of 31.1±19.8 months, this number rose to 6 patients (27.3%), all of them died by respiratory cause and had NSIP pattern. No differences were found in age, last FVC, therapy and time of disease evolution between those who died and the others.Conclusion:Our study showed that a small proportion of IPAF patients had a progressive phenotype and the NSIP pattern seemed to be a poor prognosis factor for survival.References:[1]Ito Y, Arita M, Kumagai S, et al. Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features. BMC Pulm Med 2017; 17:111 10.1186/s12890-017-0453-zDisclosure of Interests:None declared

scholarly journals AB0369 EFFICACY AND SAFETY OF RITUXIMAB ORIGINATOR AND BIOSIMILAR IN PRIMARY SJÖGREN’S SYNDROME IN A REAL-LIFE SETTING

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1485.3-1485
Author(s):  
F. Carubbi ◽  
A. Alunno ◽  
P. Cipriani ◽  
V. Pavlych ◽  
C. DI Muzio ◽  
...  
Keyword(s):  
Disease Activity ◽  
Real Life ◽  
Primary Sjögren’S Syndrome ◽  
Efficacy And Safety ◽  
Research Support ◽  
Treatment Groups ◽  
Real Life Setting ◽  
Patient Reported ◽  
Time Point

Background:Over the last 2 decades rituximab (RTX) has been widely used, albeit off-label, in primary Sjögren’s syndrome (pSS). Several studies reported that B-lymphocyte depletion with RTX is effective in this disease not only by reducing disease activity but also by affecting the inflammation and the lymphoid organization that occur in target tissues. With the recent release of several RTX biosimilars (bRTX) on the market, the demonstration of their interchangeability with RTX originator (oRTX) is required.Objectives:To compare efficacy and safety of oRTX and bRTX in pSS patients in a real-life setting.Methods:Clinical records of pSS patients referring to a tertiary rheumatology clinic were retrospectively evaluated. Patients having received at least 2 courses of either oRTX or bRTX (1000 mg IV infusion, repeated after 2 weeks -1 course- and the course repeated after 24 weeks) with complete data at baseline and after 3, 6, 9 and 12 months of treatment were enrolled. Disease activity was assessed with the EULAR SS disease activity index (ESSDAI) and its clinical version without the biological domain (ClinESSDAI). Patient-reported symptoms were assessed with the EULAR SS Patient Reported Index (ESSPRI).Results:Seven patients that received oRTX and 7 patients that received bRTX were enrolled. Baseline clinical features, including ESSDAI and ESSPRI were similar in the 2 treatment groups. Both compounds significantly reduced ESSDAI and ESSPRI as early as 3 months and no difference between the groups was observed at any time point (Figure 1). Of interest, ESSDAI slowly decreased until month 6 when the most pronounced reduction was observed. Conversely, ESSPRI dropped to its lowest values already at month 3. With regard to safety, at 12 months of follow-up no adverse event was observed in any of the treatment groups.Conclusion:At 12 months of follow-up, oRTX and bRTX display similar efficacy and safety profiles. The improvement of patient reported outcomes is faster than the improvement of disease activity with both compounds. Our data support interchangeability of oRTX and bRTX in pSS.References:[1]Carubbi F et al. Arthritis Res Ther. 2013;15(5):R172[2]Carubbi F et al. Lupus. 2014;23(13):1337-49Figure 1 ESSDAI and ESSPRI values at every time point in the 2 treatment groups. Asterisks indicate p values <0.05 compared to the other treatment group at the same time pointDisclosure of Interests:Francesco Carubbi Speakers bureau: Francesco Carubbi received speaker honoraria from Abbvie and Celgene outside this work., Alessia Alunno: None declared, Paola Cipriani Grant/research support from: Actelion, Pfizer, Speakers bureau: Actelion, Pfizer, Viktoriya Pavlych: None declared, claudia di muzio: None declared, Roberto Gerli: None declared, Roberto Giacomelli Grant/research support from: Actelion, Pfizer, Speakers bureau: Abbvie, Roche, Actelion, BMS, MSD, Ely Lilly, SOBI, Pfizer

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.

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