Cells Tissues Organs. 2021 Apr 26:1-18. doi: 10.1159/000514579. Online ahead of print.

ABSTRACT

Mucociliary epithelia are composed of multiciliated, secretory, and stem cells and line various organs in vertebrates such as the respiratory tract. By means of mucociliary clearance, those epithelia provide a first line of defense against inhaled particles and pathogens. Mucociliary clearance relies on the correct composition of cell types, that is, the proper balance of ciliated and secretory cells. A failure to generate and to maintain correct cell type composition and function results in impaired clearance and high risk to infections, such as in congenital diseases (e.g., ciliopathies) as well as in acquired diseases, including asthma, chronic obstructive pulmonary disease (COPD), and idiopathic pulmonary fibrosis (IPF). While it remains incompletely resolved how precisely cell types are specified and maintained in development and disease, many studies have revealed important mechanisms regarding the signaling control in mucociliary cell types in various species. Those studies not only provided insights into the signaling contribution to organ development and regeneration but also highlighted the remarkable plasticity of cell identity encountered in mucociliary maintenance, including frequent trans-differentiation events during homeostasis and specifically in disease. This review will summarize major findings and provide perspectives regarding the future of mucociliary research and the treatment of chronic airway diseases associated with tissue remodeling.

PMID:33902038 | DOI:10.1159/000514579

Biomed Pharmacother. 2021 Apr 23;139:111500. doi: 10.1016/j.biopha.2021.111500. Online ahead of print.

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is the most common fatal interstitial lung disease, with limited therapeutic options. The abnormal and uncontrolled differentiation and proliferation of fibroblasts have been confirmed to play a crucial role in driving the pathogenesis of IPF. Therefore, effective and well-tolerated antifibrotic agents that interfere with fibroblasts would be an ideal treatment, but no such treatments are available. Remarkably, we found that dopamine (DA) receptor D1 (D1R) and DA receptor D2 (D2R) were both upregulated in myofibroblasts in lungs of IPF patients and a bleomycin (BLM)-induced mouse model. Then, we explored the safety and efficacy of DA, fenoldopam (FNP, a selective D1R agonist) and sumanirole (SMR, a selective D2R agonist) in reversing BLM-induced pulmonary fibrosis. Further data showed that DA receptor agonists exerted potent antifibrotic effects in BLM-induced pulmonary fibrosis by attenuating the differentiation and proliferation of fibroblasts. Detailed pathway analysis revealed that DA receptor agonists decreased the phosphorylation of Smad2 induced by TGF-β1 in primary human lung fibroblasts (PHLFs) and IMR-90 cells. Overall, DA receptor agonists protected mice from BLM-induced pulmonary fibrosis and may be therapeutically beneficial for IPF patients in a clinical setting.

PMID:33901873 | DOI:10.1016/j.biopha.2021.111500

Exp Lung Res. 2021 Apr 24:1-9. doi: 10.1080/01902148.2021.1916650. Online ahead of print.

ABSTRACT

BACKGROUND AND OBJECTIVES: Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disease. An increased expression of somatostatin receptor subtype 2 in patients with IPF was identified and lung fibroblasts expressed somatostatin receptors in vitro. In addition, somatostatin analogue inhibits the expression of transforming growth factor-β, insulin-like growth factor (IGF) -1, platelet-derived growth factor, and basic fibroblast growth factor. Therefore, we examined the effects of somatostatin analogue on bleomycin-induced pulmonary fibrosis in mice. In a similar model, it has been reported that administration of high-dose somatostatin analogs suppressed acute inflammation and subsequent pulmonary fibrosis. However, it was clarified that the same effect can be obtained even at the dose used in clinical practice.

METHODS: C57BL/6 mice received a single tracheal instillation of bleomycin. After randomly allocated, mice were treated with subcutaneous injection of either normal saline or somatostatin analogue.

RESULTS: Somatostatin analogue reduced the number of neutrophils and lymphocytes in bronchoalveolar lavage (BAL) and IGF-1 level in serum and BAL fluid and attenuated weight loss. The hydroxyproline content of the lung homogenates in somatostatin analogue treatment group was significantly lower than in that of normal saline treatment group.

CONCLUSIONS: These results suggest that somatostatin analogue may attenuate pulmonary fibrosis after bleomycin treatment at the dose used in clinical practice.

PMID:33899633 | DOI:10.1080/01902148.2021.1916650

Tissue Eng Part B Rev. 2021 Apr 24. doi: 10.1089/ten.TEB.2020.0357. Online ahead of print.

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease with significant gas exchange impairment due to exaggerated extracellular matrix deposition and myofibroblast activation. IPF has no cure, and although nintedanib and pirfenidone are two approved medications for symptom management, the total treatment cost is exuberant and prohibitive to a global uninsured patient population. New therapeutic alternatives with moderate costs are needed to treat IPF. Extracellular matrix hydrogels derived from decellularized lungs are cost-effective therapeutic candidates to treat pulmonary fibrosis due to their reported antioxidant properties. Oxidative stress contributes to IPF pathophysiology by damaging macromolecules, interfering with tissue remodeling, and contributing to myofibroblast activation. Thus, preventing oxidative stress has beneficial outcomes in IPF. For this purpose, the present review describes ECM hydrogel's properties to regulate oxidative stress and tissue remodeling in idiopathic pulmonary fibrosis.

PMID:33899554 | DOI:10.1089/ten.TEB.2020.0357

Arch Bronconeumol. 2021 Mar 27:S0300-2896(21)00116-2. doi: 10.1016/j.arbres.2021.03.018. Online ahead of print.

ABSTRACT

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials.

METHODS: Spanish multicentre, observational, retrospective study of IPF patients diagnosed between 2012 and 2016, based on the ATS/ERS criteria, which presented FVC greater or equal 80% at diagnosis. Clinical and demographic characteristics, lung function, radiological pattern, treatment, and follow-up were analyzed.

RESULTS: 225 IPF patients were included, 72.9% were men. The mean age was 69.5 years. The predominant high-resolution computed tomography (HRCT) pattern was consistent usual interstitial pneumonia (UIP) (51.6%). 84.7% of patients presented respiratory symptoms (exertional dyspnea and/or cough) and 33.33% showed oxygen desaturation below 90% in the 6min walking test (6MWT). Anti-fibrotic treatment was initiated at diagnosis in 55.11% of patients. Median FVC was 89.6% (IQR 17) and 58.7% of patients had a decrease of diffusion lung capacity for carbon monoxide (DLCO) below 60% of theoretical value; most of them presented functional progression (61.4%) and higher mortality at 3 years (20.45%). A statistically significant correlation with the 3-years mortality was observed between DLCO <60% and consistent UIP radiological pattern.

CONCLUSIONS: Patients with preserved FVC but presenting UIP radiological pattern and moderate-severe DLCO decrease at diagnosis associate an increased risk of progression, death or lung transplantation. Therefore, in these cases, preserved FVC would not be representative of early or mild IPF.

PMID:33895005 | DOI:10.1016/j.arbres.2021.03.018

Am J Pathol. 2021 Apr 21:S0002-9440(21)00161-9. doi: 10.1016/j.ajpath.2021.04.006. Online ahead of print.

ABSTRACT

Pulmonary fibrosis (PF) can arise from unknown causes as in idiopathic pulmonary fibrosis (IPF), or as a consequence of infections including severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Current treatments for PF slow, but do not stop disease progression. We report that treatment with a RUNX1 inhibitor (Ro24-7429), previously found to be safe, though ineffective, as a Tat inhibitor in patients with HIV, robustly ameliorates lung fibrosis and inflammation in the bleomycin-induced PF mouse model. RUNX1 inhibition blunted fundamental mechanisms downstream pathological mediators of fibrosis and inflammation including TGF-β1 and TNF-α in cultured lung epithelial cells, fibroblasts, and vascular endothelial cells indicating pleiotropic effects. RUNX1 inhibition also reduced the expression of ACE2 and FURIN, host proteins critical for SARS-CoV-2 infection, in mice and in vitro. A subset of human lungs with SARS-CoV-2 infection overexpress RUNX1. These data suggest that RUNX1 inhibition via repurposing of Ro24-7429 may be beneficial for PF and to battle SARS-CoV-2, by reducing expression of viral mediators and by preventing respiratory complications.

PMID:33894177 | DOI:10.1016/j.ajpath.2021.04.006

Respir Res. 2021 Apr 23;22(1):120. doi: 10.1186/s12931-021-01727-7.

ABSTRACT

BACKGROUND: The differential diagnosis fibrotic hypersensitivity pneumonitis (HP) versus idiopathic pulmonary fibrosis (IPF) is important but challenging. Recent diagnostic guidelines for HP emphasize including multidisciplinary discussion (MDD) in the diagnostic process, however MDD is not comprehensively available. We aimed to establish the diagnostic accuracy and prognostic validity of a previously proposed HP diagnostic algorithm that foregoes MDD.

METHODS: We tested the algorithm in patients with an MDD diagnosis of fibrotic HP or IPF (case control study) and determined diagnostic test performances for diagnostic confidences of ≥ 90% and ≥ 70%. Prognostic validity was established using Cox proportional hazards models.

RESULTS: Thirty-one patients with fibrotic HP and 50 IPF patients were included. The algorithm-derived ≥ 90% confidence level for HP had high specificity (0.94, 95% confidence interval [CI] 0.83-0.99), but low sensitivity (0.35 [95%CI 0.19-0.55], J-index 0.29). Test performance was improved for the ≥ 70% confidence level (J-index 0.64) with a specificity of 0.90 (95%CI 0.78-0.97), and a sensitivity of 0.74 (95%CI 0.55-0.88). MDD fibrotic HP diagnosis was strongly associated with lower risk of death (adjusted hazard ratio [HR] 0.10 [0.01-0.92], p = 0.04), whereas the algorithm-derived ≥ 70% and ≥ 90% confidence diagnoses were not significantly associated with survival (adjusted HR 0.37 [0.07-1.80], p = 0.22, and adjusted HR 0.41 [0.05-3.25], p = 0.39, respectively).

CONCLUSION: The algorithm-derived ≥ 70% diagnostic confidence had satisfactory test performance for MDD-HP diagnosis, with insufficient sensitivity for ≥ 90% confidence. The lowest risk of death in the MDD-derived HP diagnosis validates the reference standard and suggests that a diagnostic algorithm not including MDD, might not replace the latter.

PMID:33892724 | DOI:10.1186/s12931-021-01727-7

BMC Vet Res. 2021 Apr 23;17(1):171. doi: 10.1186/s12917-021-02879-w.

ABSTRACT

BACKGROUND: Pulmonary hypertension (PH) is a known co-morbidity in West Highland white terriers (WHWTs) affected with canine idiopathic pulmonary fibrosis (CIPF). The pulmonary vein-to-right pulmonary artery ratio (PV/PA) has recently been described for the detection of pre-capillary PH in dogs. The objective of the present study was to estimate the prevalence of PH at diagnostic, in WHWTs affected with CIPF, by using PV/PA, in comparison with a group of healthy breed-matched controls (CTRLs). Additional study objective was to explore whether the presence of PH at initial diagnosis of CIPF impacted survival time in dogs treated with sildenafil.

RESULTS: Twenty-five client-owned WHWTs presented with CIPF and 19 CTRLs were included in the study. PV/PA in either two-dimensional mode (2D) or time-motion mode or both were measured from cineloops in each dog. Dogs were classified according to PV/PA value into non/mild PH (PV/PA measured in 2D ≥ 0.7) or moderate/severe PH (PV/PA < 0.7). Survival data of WHWTs affected with CIPF were extracted from medical record to assess association between presence of PH at diagnosis and outcome. 60 % overall prevalence for moderate/severe PH was estimated in this cohort of WHWTs presented with CIPF vs. 5 % in CTRLS (P = 0.0002). The presence of moderate/severe PH at initial presentation was not associated with survival.

CONCLUSIONS: Results of the present study confirm a high prevalence of PH at diagnosis in WHWTs affected with CIPF and highlight the utility of PV/PA as a non-invasive surrogate for assessment of PH in this population.

PMID:33892687 | DOI:10.1186/s12917-021-02879-w

Am J Respir Crit Care Med. 2021 Apr 23. doi: 10.1164/rccm.202103-0737ED. Online ahead of print.

NO ABSTRACT

PMID:33891825 | DOI:10.1164/rccm.202103-0737ED

ChemMedChem. 2021 Apr 22. doi: 10.1002/cmdc.202100064. Online ahead of print.

ABSTRACT

Heat shock protein 47 (HSP47), a collagen-specific molecular chaperone, is causally related to fibrotic diseases, including idiopathic pulmonary fibrosis. The identification of compounds that interfere with the HSP47-collagen interaction is essential for the development of relevant therapeutics. Herein, we prepared human HSP47 as a soluble fusion protein expressed in E. coli and established an assay system for HSP47 inhibitor screening. We screened a natural and synthetic compound library established at Nagasaki University. Among 1023 compounds, 13 exhibited inhibitory activity against human HSP47, of which three inhibited its function in a dose-dependent manner. Epigallocatechin-3-O-gallate, one of these three compounds, is a typical polyphenol compound derived from tea leaves. Structurally related compounds were synthesized and examined for their activity, revealing a hydroxyl group at A-ring position 5 as important for its activity. The present findings provide valuable insight for the development of natural product-derived therapeutics for fibrotic diseases, including idiopathic pulmonary fibrosis.

PMID:33890415 | DOI:10.1002/cmdc.202100064

Respirology. 2021 Apr 22. doi: 10.1111/resp.14068. Online ahead of print.

NO ABSTRACT

PMID:33890384 | DOI:10.1111/resp.14068

J Med Imaging (Bellingham). 2021 May;8(3):031903. doi: 10.1117/1.JMI.8.3.031903. Epub 2021 Apr 19.

ABSTRACT

Purpose: The purpose of our study was to combine differences in radiomic features extracted from lung regions in the computed tomography (CT) scans of patients diagnosed with idiopathic pulmonary fibrosis (IPF) to identify associations with genetic variations and patient survival. Approach: A database of CT scans and genomic data from 169 patients diagnosed with IPF was collected retrospectively. Six region-of-interest pairs (three per lung, positioned posteriorly, anteriorly, and laterally) were placed in each of three axial CT sections for each patient. Thirty-one features were used in logistic regression to classify patients' genetic mutation status; classification performance was evaluated through the area under the receiver operating characteristic (ROC) curve [average area under the ROC curve (AUC)]. Kaplan-Meier (KM) survival curve models quantified the ability of each feature to differentiate between survival curves based on feature-specific thresholds. Results: Nine first-order texture features and one fractal feature were correlated with TOLLIP-1 (rs4963062) mutations (AUC: 0.54 to 0.74), and five Laws' filter features were correlated with TOLLIP-2 (rs5743905) mutations (AUC: 0.53 to 0.70). None of the features analyzed were found to be correlated with MUC5B mutations. First-order and fractal features demonstrated the greatest discrimination between KM curves. Conclusions: A radiomics approach for the correlation of patient genetic mutations with image texture features has potential as a biomarker. These features also may serve as prognostic indicators using a survival curve modeling approach in which the combination of radiomic features and genetic mutations provides an enhanced understanding of the interaction between imaging phenotype and patient genotype on the progression and treatment of IPF.

PMID:33889657 | PMC:PMC8054271 | DOI:10.1117/1.JMI.8.3.031903

Respir Investig. 2021 Apr 19:S2212-5345(21)00049-6. doi: 10.1016/j.resinv.2021.03.006. Online ahead of print.

ABSTRACT

BACKGROUND: Lung transplantation (LTx) is the last resort for patients who fail to respond to drug therapy and progress to advanced idiopathic interstitial pneumonias (IIPs). However, more than one-third of patients registered for LTx face despair because of rapid disease progression and donor shortage. This study aimed to identify the risk factors of waitlist mortality in LTx candidates with IIPs and investigate the association of anti-fibrotic therapy with waitlist mortality.

METHODS: We retrospectively investigated 56 patients with IIPs, including 29 patients with idiopathic pulmonary fibrosis (51.7%) and 11 patients with idiopathic pleuroparenchymal fibroelastosis (19.6%), registered for LTx at Fukuoka University Hospital between January 2006 and June 2020. The risk factors affecting transplantation-censored survival were evaluated.

RESULTS: The waitlist mortality rate of patients with nonspecific interstitial pneumonia was significantly lower than that of others. Multivariate survival analysis using Cox's model identified a history of pneumothorax (P = 0.029) and short 6-min walk distance (6MWD) (P = 0.012) to be significant variables affecting waitlist mortality. Patients receiving anti-fibrotic therapy (n = 27, 48.2%) had a lower risk of pneumothorax (P = 0.017) and their 6MWD was longer than that of non-therapy patients (P < 0.001). The waitlist mortality rate of patients on anti-fibrotic therapy was significantly lower (P = 0.012).

CONCLUSIONS: History of pneumothorax and short 6MWD were independent predictors of waitlist mortality in LTx candidates with IIPs. The anti-fibrotic therapy may potentially reduce mortality in patients with IIPs on the waiting list for LTx.

PMID:33888448 | DOI:10.1016/j.resinv.2021.03.006

Am J Pathol. 2021 Apr 19:S0002-9440(21)00154-1. doi: 10.1016/j.ajpath.2021.04.003. Online ahead of print.

ABSTRACT

Increased apoptosis sensitivity of alveolar type 2 (ATII) cells and increased apoptosis resistance of (myo)fibroblasts, the apoptosis paradox, is believed to contribute importantly to the pathogenesis of idiopathic pulmonary fibrosis (IPF). The mechanism underlying the apoptosis paradox in IPF lungs, however, is unclear. Aging is the greatest risk factor for IPF. In this study, we show for the first time that ATII cells from old mice are more sensitive, whereas fibroblasts from old mice are more resistant, to apoptotic challenges, compared to the corresponding cells from young mice. We also found that the expression of plasminogen activator inhibitor1 (PAI-1), an important profibrogenic mediator, is significantly increased in both ATII cells and lung fibroblasts from aged mice. In vitro studies using PAI-1 siRNA and active PAI-1 protein indicate that PAI-1 promotes ATII cell apoptosis but protects fibroblasts from apoptosis, likely through dichotomous regulation of p53 expression. In vivo studies further show that deletion of PAI-1 in adult mice reduces p53, p21, and Bax proteins as well as apoptosis sensitivity in ATII cells but the opposite effects in lung fibroblasts, associated with an attenuation of lung fibrosis after bleomycin challenge. As PAI-1 is upregulated in both ATII cells and fibroblasts in IPF, the results suggest that increased PAI-1 may underlie the apoptosis paradox of ATII cells and fibroblasts in IPF lungs.

PMID:33887217 | DOI:10.1016/j.ajpath.2021.04.003

Clin Respir J. 2021 Apr 21. doi: 10.1111/crj.13380. Online ahead of print.

NO ABSTRACT

PMID:33884764 | DOI:10.1111/crj.13380

Eur J Pharm Biopharm. 2021 Apr 18:S0939-6411(21)00099-0. doi: 10.1016/j.ejpb.2021.03.017. Online ahead of print.

ABSTRACT

Idiopathic pulmonary fibrosis (IPF) is a debilitating and fatal condition that causes severe scarring of the lungs. While the pathogenesis of IPF continues to be extensively studied and several factors have been considered, an exact cause has yet to be established. With inadequate treatment options and no cure available, overall disease prognosis is still poor. Existing oral therapies, pirfenidone and nintedanib, may attempt to improve the patients' quality of life by mitigating symptoms and slowing disease progression, however chronic doses and systemic deliveries of these drugs can lead to severe side effects. The lack of effective treatment options calls for further investigation of restorative as well as additional palliative therapies for IPF. Nanoparticle-based sustained drug delivery strategies can be utilized to ensure targeted delivery for site-specific treatment as well as long-acting therapy, improving overall patient compliance. This review provides an update on promising strategies for the delivery of anti-fibrotic agents, along with an overview of key therapeutic targets as well as relevant emerging therapies currently being evaluated for IPF treatment.

PMID:33882301 | DOI:10.1016/j.ejpb.2021.03.017

Expert Opin Drug Saf. 2021 Apr 21. doi: 10.1080/14740338.2021.1921143. Online ahead of print.

ABSTRACT

INTRODUCTION: The approval of antifibrotic agents nintedanib and pirfenidone revolutionized the management of idiopathic pulmonary fibrosis (IPF). These treatments showed acceptable tolerability in randomized clinical trials, however they have been associated to a spectrum of potential side effects which require careful assessment of risks and benefits in the individual patient before commencing and during antifibrotic therapy.

AREAS COVERED: the accrued evidence on safety of nintedanib and pirfenidone is summarized, from the first randomized clinical trials to the open-label extension studies and post-marketing clinical experiences which helped clarify the long-term tolerability of these drugs.

EXPERT OPINION: the data collected over the last years confirmed the comparable tolerability profile of nintedanib and pirfenidone. The physician's assessment of expected side effects may help decide the optimal first line therapy for the individual patient. Patient's counselling during treatment remains essential to manage emerging adverse events and eventually inform the decision of drug discontinuation.

PMID:33881959 | DOI:10.1080/14740338.2021.1921143

Br J Radiol. 2021 Apr 21:20200944. doi: 10.1259/bjr.20200944. Online ahead of print.

ABSTRACT

In patients with idiopathic pulmonary fibrosis (IPF), there is an urgent need of biomarkers which can predict disease behaviour or response to treatment. Most published studies report results based on continuous data which can be difficult to apply to individual patients in clinical practice. Having antifibrotic therapies makes it even more important that we can accurately diagnose and prognosticate in IPF patients. Advances in computer technology over the past decade have provided computer-based methods for objectively quantifying fibrotic lung disease on high-resolution CT of the chest with greater strength than visual CT analysis scores. These computer-based methods and, more recently, the arrival of deep learning-based image analysis might provide a response to these unsolved problems. The purpose of this commentary is to provide insights into the problems associated with visual interpretation of HRCT, describe of the current technologies used to provide quantification of disease on HRCT and prognostication in IPF patients, discuss challenges to the implementation of this technology and future directions.

PMID:33881923 | DOI:10.1259/bjr.20200944

Adv Respir Med. 2021 Apr 21. doi: 10.5603/ARM.a2021.0012. Online ahead of print.

ABSTRACT

INTRODUCTION: Bronchoalveolar lavage (BAL) is useful for diagnosing diffuse lung disease and excluding other conditions. However, acute exacerbations (AEs) are recognized as important complications of BAL in patients with idiopathic pulmonary fibrosis (IPF). This study aimed to identify risk factors for BAL-induced AEs in patients with IPF.

MATERIAL AND METHODS: We retrospectively analyzed the data of 155 patients with suspected IPF who had undergone BAL between January 2013 and December 2018. BAL-related AE was defined as the development of AE within 30 days after the procedure. We compared clinical features and parameters between patients with AE (AE group) and without AE (non-AE group). We also reviewed the relevant reported literature.

RESULTS: Among the 155 patients, 5 (3.2%) developed AE within 30 days after BAL. The average duration from BAL to AE onset was 7.8 days (2-16 days). Results from the univariate analysis revealed PaO2 < 75 mm Hg (p = 0.036), neutrophil content in BAL ≥ 7% (p = 0.0061), %DLCO < 50% (p = 0.019), Gender-Age-Physiology (GAP) stage III (p = 0.034), and BAL recovery rates < 30% (p < 0.001) as significant risk factors for post-BAL AE. All five patients who developed AE recovered and were discharged.

CONCLUSIONS: Disease severity, high neutrophil levels in BAL, and poor BAL recovery rates may be risk factors for BAL-induced AEs.

PMID:33881153 | DOI:10.5603/ARM.a2021.0012

Med Lav. 2021 Apr 20;112(2):115-122. doi: 10.23749/mdl.v112i2.10473.

ABSTRACT

INTRODUCTION: Idiopathic pulmonary fibrosis (IPF) and asbestosis are pulmonary interstitial diseases that may present overlapping clinical aspects in the full-blown phase of the disease. For both clinical entities the gold standard for diagnosis is histological examination, but its execution poses ethical problems, especially when performed for preventive or forensic purposes.

OBJECTIVE: To evaluate the application of internationally accepted clinical, anamnestic and radiological criteria for differential diagnosis between asbestosis and IPF, and to assess the ability to discriminate between the two diseases. Even if clinically similar, the two diseases present extremely different prognostic and therapeutic perspectives.

METHODS: Two clinical cases of IPF are reported, in which the differential diagnosis was made by studying occupational exposure to asbestos, the onset and progression of clinical symptoms, and the identification of specific radiological elements by means of chest High Resolution Computed Tomography (HRCT).

RESULTS: The diagnosis of IPF could be made on the basis of the absence of significant exposure to asbestos, the early onset and rapid progression of dyspnea and restrictive ventilatory defects, in association with a pulmonary radiological pattern characterized by peculiar elements such as honeycombing.

DISCUSSION: The diagnostic procedure adopted to make a differential diagnosis with asbestosis provides practical clinical elements facilitating the differentiation between the two forms of pulmonary fibrosis, a fundamental aspect of the activity of the occupational physician.

PMID:33881005 | DOI:10.23749/mdl.v112i2.10473