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Background and objective: In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods: Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results: A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49–2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83–2.44, P = 0.201 for disease progression). Conclusion: In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

Introduction: In recent decades, the primary focus of pharmaceutical research in interstitial lung diseases (ILD) has been on idiopathic pulmonary fibrosis (IPF). Recently, pharmaceutical development has also focused on other forms of ILDs, including connective tissue diseases associated ILD, fibrotic hypersensitivity pneumonitis, and sarcoidosis. Areas Covered: The authors summarize the advances in pharmacotherapy for the treatment of ILD. Specifically, the authors review the most recent studies and discuss the most recent research findings and future prospects. Expert opinion: Data collected over the past years have confirmed the efficacy of antifibrotic drugs on slowing disease progression in IPF. The usual strategy for CTD-ILD management is represented by the combined use of corticosteroids and immunosuppressive agents. There is an urgent need for new target therapies. The concept of progressive fibrosing ILD has emerged in the ILD community in recent years, which has led to grouping several diseases with a common disease behavior to find an effective treatment. At present, selecting the best therapy in ILDs should be reasonably performed on a case-by-case basis through a multidisciplinary team discussion in tertiary ILD centers, taking into consideration patients’ symptoms, lung functional trends, and radiological changes.

Introduction: The novel coronavirus SARS-Cov-2 can infect the respiratory tract causing a spectrum of disease varying from mild to fatal pneumonia, and known as COVID-19. Ongoing clinical research is assessing the potential for long-term respiratory sequelae in these patients. We assessed the respiratory function in a cohort of patients after recovering from SARS-Cov-2 infection, stratified according to PaO2/FiO2 (p/F) values. Method: Approximately one month after hospital discharge, 86 COVID-19 patients underwent physical examination, arterial blood gas (ABG) analysis, pulmonary function tests (PFTs), and six-minute walk test (6MWT). Patients were also asked to quantify the severity of dyspnoea and cough before, during, and after hospitalization using a visual analogic scale (VAS). Seventy-six subjects with ABG during hospitalization were stratified in three groups according to their worst p/F values: above 300 (n = 38), between 200 and 300 (n = 30) and below 200 (n = 20). Results: On PFTs, lung volumes were overall preserved yet, mean percent predicted residual volume was slightly reduced (74.8 ± 18.1%). Percent predicted diffusing capacity for carbon monoxide (DLCO) was also mildly reduced (77.2 ± 16.5%). Patients reported residual breathlessness at the time of the visit (VAS 19.8, p < 0.001). Patients with p/F below 200 during hospitalization had lower percent predicted forced vital capacity (p = 0.005), lower percent predicted total lung capacity (p = 0.012), lower DLCO (p < 0.001) and shorter 6MWT distance (p = 0.004) than patients with higher p/F. Conclusion: Approximately one month after hospital discharge, patients with COVID-19 can have residual respiratory impairment, including lower exercise tolerance. The extent of this impairment seems to correlate with the severity of respiratory failure during hospitalization.

Introduction: The last two decades witnessed an increasing number of well-designed late phase trials in patients with Idiopathic Pulmonary Fibrosis (IPF), leading to the approval of the first effective therapies for these patients, pirfenidone and nintedanib. Currently, novel putative agents for the treatment of IPF are being tested in phase III trials, possibly marking a new breakthrough in IPF management. Areas covered: In this review, the available evidence on completed phase III trials in IPF is summarized, from the past failures of immunosuppressive and anti-inflammatory agents, anticoagulants and endothelin-receptor antagonists to the positive results of the antifibrotic treatments that revolutionized IPF therapeutic landscape. Literature search was performed using Medline and Clinicaltrials.org databases (1999–2020). Expert opinion: In the relatively young history of pharmaceutical research in IPF, most phase III trials provided disappointing results, however the lessons learned helped paving the way to the success of the first therapies capable of modifying the natural history of this deadly disease. To date, the conduction of robustly designed phase III trials on novel drugs remains crucial to pursue the goal of halting disease progression in these patients, using a therapeutic approach that should become more and more tailored to the individual.

Introduction: The two available therapies for idiopathic pulmonary fibrosis (IPF), pirfenidone and nintedanib, slow down but do not halt IPF progression. As such, in the last few years several agents with specific molecular targets have been investigated to find a cure forIPF. Pamrevlumab, a recombinant human antibody that binds to connective tissue growth factor (CTGF) has emerged as a potential therapy for IPF and has advanced to phase 3 clinical trials. Areas covered: The authors offer a backdrop to the current IPF treatment market and describe the chemistry, pharmacokinetics and pharmacodynamics of pamrevlumab. They summarize the preclinical and early clinical evidence on pamrevlumab and propose ways of progressing this agent further as a potential IPF treatment. Expert opinion: Pamrevlumab was effective and safe in patients in a placebo-controlled phase 2 trial, demonstrating its potential to become an alternative therapeutic option for IPF; however, the feasibility of intravenous administration in clinical practice may be a hurdle to its use as a first-line treatment. Further studies are necessary to assess its effects when administered with pirfenidone or nintedanib and this could open up a new era of combined therapeutic approaches for IPF.

Early diagnosis of idiopathic pulmonary fibrosis (IPF) is definitely a challenge. Several studies have been investigating how to achieve this goal. IPF is the most common type of idiopathic interstitial pneumonia, the median age at diagnosis is approximately 65 years and it's more common in smokers and males [ 1 ]. To date, there are only two therapeutic options available (pirfenidone and nintedanib) that have shown to be effectively slowing disease progression in terms of forced vital capacity (FVC) [ 2 ] with a further decrease in the risk of acute exacerbations for nintedanib [ 3 ]. Regardless of the disease severity, the delay between first respiratory symptoms and referral to a tertiary care centre could be years and this ‘waiting’ leads to an increase in mortality [ 4 ]. Furthermore, the absence of accurate short-term indicators of therapeutic response leaves clinicians with uncertainty regarding management. Reduced survival time has been associated with various factors such as advanced age, severe physiological impairment, low body-mass index, radiological extent and severity of fibrosis determined by chest high-resolution computed tomography (HRCT), presence of comorbidities including pulmonary hypertension (PH), emphysema and bronchogenic cancer. Several risk prediction models have been developed in order to improve clinicians’ ability to predict prognosis in IPF and to modify the natural course of the disease, however accurate prognostication in patients with IPF is challenging due to the highly variable natural course of the disease [ 5 ]. Formulating a diagnosis of IPF at an early stage and increasing prognostic accuracy are fundamental strategies to optimize clinical management and allow timely referral for lung transplantations.

Background and objectives: Evidence of mediastinal Lymph Node Enlargement (LNE) on CT scan is a common finding in idiopathic pulmonary fibrosis (IPF). We sought to investigate whether the involvement of mediastinal lymph nodes is associated with accelerated disease progression, and explored the changes occurring in mediastinal lymph nodes during the radiological follow up of these patients. Methods: This retrospective study included IPF patients referred to a single ILD centre in Italy. A consensus-based assessment of mediastinal LNE on chest CT scan was performed by two thoracic radiologists. Kaplan-Meier curves and multivariate Cox proportional hazards regression were used to assess hazard ratios for mortality and disease progression (defined as categorical FVC decline ≥10%). The annualized rates of change in functional parameters for each patient were calculated using mixed linear models. Results: The study population consisted of 152 IPF patients, of whom 135 (89%) received antifibrotic treatment for IPF during the study follow up. Patients having evidence of 3 or more enlarged mediastinal lymph nodes on baseline CT scan showed increased rates of mortality (HR 5.03, 95% CI 1.86-13.62, p ≤ 0.001) and significant disease progression (HR 2.99, 95% CI 1.22-7.33, p = 0.17) as compared to patients without LNE, after adjusting for GAP stage. Among 62 patients with LNE who underwent a follow up CT scan of the chest and received antifibrotic treatment, 57 (92%) maintained evidence mediastinal LNE over time. Conclusions: Diffuse mediastinal lymph node involvement predicts clinically meaningful functional deterioration in patients with IPF.

Fibrotic hypersensitivity pneumonitis is a complex interstitial lung disease that is not entirely understood. In its chronic and fibrotic form, hypersensitivity pneumonitis is one of the main mimickers of idiopathic pulmonary fibrosis (IPF). Distinguishing between these two conditions is challenging but is of particular clinical relevance. Two approved therapies are available for IPF, and a considerable number of clinical trials are now exploring newer pharmacological options. This impressive research effort is a consequence of new pathogenetic understanding, updated diagnostic criteria and a long history of pharmacological trials. Conversely, current knowledge gaps on pathogenesis of chronic hypersensitivity pneumonitis, coupled with lack of validated diagnostic criteria, make the management of this disease an unsolved clinical challenge. This also reflects the paucity of therapeutic clinical trials in this field. In this review, we describe the current evidence and the possible future options to approach this complex disease.

No abstract available

Background: Stenotrophomonas maltophilia is a bacteria whose role in patients with cystic fibrosis bronchiectasis has been previously studied; little is known about its role in non-CF bronchiectasis. Aim: Investigate the risk factors for S. Maltophilia acquisition and its clinical impact on bronchiectasis patients. Design: A retrospective observational cohort study enrolling patients attending the Bronchiectasis Clinic at the Royal Infirmary of Edinburgh, Scotland, UK. Methods: 167 bronchiectasis patients undergoing intravenous antibiotic therapy were selected and divided according to single or chronic S. Maltophilia isolation in sputum. The risk factors and prognostic impact was studied. Results: Single isolation was independently associated with lower baseline % predicted FEV1 (OR 0.98; 95%CI 0.970-1,044; p = 0.025) and with less radiological involvement (OR 0.379; 95%CI 0.175-0.819; p = 0.01). Chronic isolation was associated with the number of intravenous antibiotic courses in the year before and after the first isolation (OR 1.2; 95%CI 1.053-1.398; p = 0.007) and with the absence of P. Aeruginosa colonisation (OR 0.207; 95%CI 0.056-0.764; p = 0.02). In the chronic isolation group, there were more exacerbations and more need of intravenous antibiotics in the year after the first isolation. Conclusions: Poor lung function is the main independent risk factor for single isolation of S. maltophilia. For chronic colonisation, the main independent risk factor is the number of intravenous antibiotic courses and the absence of P. aeruginosa chronic colonisation. Only when chronically present, S. maltophilia had a clinical impact with more exacerbations.

Background and objective: In clinical practice, a working diagnosis of IPF may be performed to provide effective antifibrotic treatment to patients who cannot undergo SLB. In this study, we compared the disease course across IPF diagnostic categories in a real-life clinical setting to clarify the appropriateness of a working diagnosis of IPF and treatment initiation in these patients. Methods: Longitudinal data from IPF patients receiving antifibrotic treatment (pirfenidone or nintedanib) were retrospectively collected at three tertiary centres in Italy. Univariate and multivariate analyses were performed to compare time to death and to a composite endpoint of disease progression between two diagnostic subgroups, that is, patients with UIP on HRCT and/or SLB, and patients with possible UIP and no histological confirmation. Results: A total of 249 IPF patients were included in the analysis. Among patients with a possible UIP pattern on HRCT, 41 (55%) were prescribed antifibrotic treatment (either nintedanib or pirfenidone) despite absence of histological confirmation. This group demonstrated similar mortality and disease progression as compared to patients with a definite diagnosis of IPF as per diagnostic guidelines (log-rank test P = 0.771 and P = 0.139, respectively). Such findings were confirmed on multivariate analysis (HR: 1.19, 95% CI: 0.49–2.89, P = 0.7 for death; HR: 1.42, 95% CI: 0.83–2.44, P = 0.201 for disease progression). Conclusion: In patients receiving antifibrotics following a working diagnosis of IPF, disease progression rates were similar to patients with a confident diagnosis of IPF according to consensus guidelines, supporting the rationale for treatment initiation in these patients by expert multidisciplinary teams.

Introduction: Pirfenidone and nintedanib have been the first agents demonstrating to slow down the progressive functional decline in patients with Idiopathic Pulmonary Fibrosis (IPF). Antibody-based therapies with precise molecular targets have been largely investigated over the last decade in IPF as alternative or complementary treatments, in the hope to ameliorate the relentless fibrotic process of IPF. Areas covered: In this review, we summarize the available evidence on two groups of monoclonal antibodies tested in IPF: those directed against known fibrogenic factors and matrix components, and those developed to antagonize the inflammation and immunity pathways. While the latter have failed to demonstrate any clinical efficacy in IPF so far, the anti-CTGF pamrevlumab has been recently proved to be capable of slowing down functional decline as compared to placebo, prompting further investigation. Expert opinion: Despite most trials on antibody-based therapies in IPF provided so far unsatisfying results, the therapeutic development in this field should continue to be pursued to deliver a more personalized treatment approach in the future, which is not currently offered by available treatment options. A more careful trial designing and the use of valid predictive markers of response to treatment are required to enhance effectiveness of future trials.

Introduction: Idiopathic pulmonary fibrosis (IPF) is a chronic, debilitating, fibrotic lung disease leading to respiratory failure, and ultimately to death. It is characterized by marked heterogeneity regarding its clinical course. Despite significant progress in the understanding of its pathogenesis, the course of the disease and the response to treatment of an individual patient cannot be reliably predicted today. Areas covered: Non-invasive biomarkers, in particular serum biomarkers, for the (early) diagnosis, differential diagnosis, prognosis, and prediction of therapeutic response are described. The molecules are classified according to their involvement into alveolar epithelial cell injury, fibroproliferation, and matrix remodeling as well as immune regulation. Furthermore, genetic variants of TOLLIP, MUC-5B, and other genes associated with a differential response to treatment and with the development and/or the prognosis of IPF are reported. Expert commentary: The combination of multiple biomarkers may identify comprehensive biomarker signatures in IPF patients. This is a way to apply personalized medicine approach to patient affected by IPF in order to not only improve our ability to diagnose and treat disease but also offer the potential to detect disease at an earlier stage, when it is potentially easier to treat effectively.

The continuing struggle in the field of interstitial lung diseases (ILDs) has long been how best to classify disease on the basis of etiology or shared pathogenetic mechanisms. In the era of antifibrotic therapeutics of proven efficacy, accurate diagnosis is more important than ever. The articles published in 2018 reflect this ongoing debate in the medical community. The year 2018 led to new recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF) with active efforts to do the same for chronic hypersensitivity pneumonitis (CHP). Over the past two decades, international agreement on diagnostic criteria of IPF allowed a deeper understanding of disease mechanisms to be reached and facilitated the achievement of outstanding therapeutic goals. However, continual reclassification should not restrain efforts aimed at the discovery of therapies that can be beneficial for patients with diverse forms of progressive fibrotic disease, who so desperately need interventions that improve both quality of life and lifespan. With an exciting array of new therapeutic targets emerging from basic laboratory investigations, the responsibility of balancing careful phenotyping while addressing compelling clinical needs via efficient trial design will rest on the respiratory community. Additional work highlights the need for pulmonologists to be advocates for the protection and support of their patients. Within the scope of this paper, we review the latest advances in diagnosis, management, and pathogenesis of IPF, which is still the focus of most of the published research in the field of ILD. We then revise the most relevant evidence published on non-IPF fibrotic lung disease, acknowledging that in the future more attention should be focused on finding efficacious treatments for those patients with progressive disease, regardless of specific etiologies and strict classification schemes.

Background: "Velcro-type" crackles on chest auscultation are considered a typical acoustic finding of Fibrotic Interstitial Lung Disease (FILD), however whether they may have a role in the early detection of these disorders has been unknown. This study investigated how "Velcro-type" crackles correlate with the presence of distinct patterns of FILD and individual radiologic features of pulmonary fibrosis on High Resolution Computed Tomography (HRCT). Methods: Lung sounds were digitally recorded from subjects immediately prior to undergoing clinically indicated chest HRCT. Audio files were independently assessed by two chest physicians and both full volume and single HRCT sections corresponding to the recording sites were extracted. The relationships between audible "Velcro-type" crackles and radiologic HRCT patterns and individual features of pulmonary fibrosis were investigated using multivariate regression models. Results: 148 subjects were enrolled: bilateral "Velcro-type" crackles predicted the presence of FILD at HRCT (OR 13.46, 95% CI 5.85-30.96, p < 0.001) and most strongly the Usual Interstitial Pneumonia (UIP) pattern (OR 19.8, 95% CI 5.28-74.25, p < 0.001). Extent of isolated reticulation (OR 2.04, 95% CI 1.62-2.57, p < 0.001), honeycombing (OR 1.88, 95% CI 1.24-2.83, < 0.01), ground glass opacities (OR 1.74, 95% CI 1.29-2.32, p < 0.001) and traction bronchiectasis (OR 1.55, 95% CI 1.03-2.32, p < 0.05) were all independently associated with the presence of "Velcro-type" crackles. Conclusions: "Velcro-type" crackles predict the presence of FILD and directly correlate with the extent of distinct radiologic features of pulmonary fibrosis. Such evidence provides grounds for further investigation of lung sounds as an early identification tool in FILD.

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive and inevitably fatal lung disease. Although the etiology and pathogenesis of IPF remain incompletely understood, two drugs (e.g., pirfenidone and nintedanib) have proven effective in slowing down functional decline and disease progression and are now approved worldwide for treatment. Yet, as outlined by the recent guideline document on treatment of IPF, every therapeutic decision needs to be tailored to the individual patient, after discussing potential benefits and pitfalls. Comorbidities, which almost invariably complicate IPF, impact significantly clinical course and prognosis of the disease, making a holistic approach to the best care for these patients. Randomized-controlled trials remain a valid choice for selected IPF patients and their completion is critically important to achieving the ultimate goal of improving both survival and quality of life of patients suffering from this devastating disease.

Background: Immunosuppressive therapy has been - until the recent release of new guidelines on diagnosis and management - the recommended treatment for idiopathic pulmonary fibrosis. However, its efficacy in patients with idiopathic pulmonary fibrosis has always been a matter of debate. Case presentation: We report the occurrence of idiopathic pulmonary fibrosis in a white man receiving chronic immunosuppressive treatment following a heart transplant. Conclusions: This case report suggests that the immune mechanisms targeted by azathioprine and cyclosporine do not play a role in the pathogenesis of idiopathic pulmonary fibrosis.

BACKGROUND Delayed diagnosis of fibrotic interstitial lung disease (ILD) is characterized by significant repercussions for management and survival. A timely assessment of “velcro-type” crackles at lung auscultation might prompt a proper diagnostic process in these patients. The accuracy of objective, computerized methods in detecting ILD from respiratory sounds has not been systematically assessed in a clinical setting. We aimed to validate automatic detection of “velcro-type” crackles by comparing the results of lung sounds analysis with chest high resolution computed tomography (HRCT) scans. METHODS Lung sounds were recorded using an electronic stethoscope (Littmann 3200) from anatomically defined sites in 56 subjects (derivation cohort) undergoing a chest HRCT scan in Modena (Italy). Three-hundred anonymized, single-layer HRCT images corresponding to the sites of sound recording were reviewed by two radiologists with expertise in ILD and scored for the signs indicating lung fibrosis. All audio recordings were analyzed by extracting a set of global acoustic features from each file and different classification algorithms were employed on a subset of the best ranked features to classify the data into two groups. The gold standard used to label the ground truth for each sound file was based on the evidence of fibrosis in the corresponding HRCT image. Two ILD physicians were also asked to independently assess the sound files for the presence of “velcro-type” crackles. The results were validated in a further cohort of 59 patients (validation cohort) undergoing chest HRCT in Parma (Italy). Three-hundred nineteen HRCT images and corresponding sound files were obtained. RESULTS In the derivation cohort, accuracy of 74.4% was achieved in automatic detection of lung sounds associated with fibrotic HRCT images. However, although specificity was high (87.3%), sensitivity was lower (48.5%). The two respiratory physicians showed comparable performance, with accuracy 71.6%, spec

Background: It has been suggested that circulating fibrocytes and endothelial cells actively participate in the intense remodelling of the pulmonary vasculature in patients with idiopathic pulmonary fibrosis (IPF). Indeed, fibrotic areas exist that have fewer blood vessels, whereas adjacent non-fibrotic tissue is highly vascularized. The number of circulating endothelial cells (CEC) and endothelial progenitor cells (EPC) might reflect the balance between vascular injury and repair. Thus, fibrocytes as well as endothelial cells could potentially be used as biomarkers of disease progression and treatment outcome. Methods: Peripheral blood samples were collected from 67 patients with a multidisciplinary diagnosis of IPF and from 45 age-matched and sex-matched healthy volunteers. Buffy coat was isolated according to standard procedures and at least 20 million cells were stained with different monoclonal antibodies for the detection of CEC, EPC and circulating fibrocytes. For the detection of CEC and EPC, cells were stained with anti-CD45, anti-CD34, anti-CD133, anti-CD14, anti-CD309 and with the viability probe Far-Red LIVE/DEAD. For the detection of circulating fibrocytes, cells were first stained with LIVE/DEAD and the following monoclonal antibodies: anti-CD3, anti-CD19, anti-CD45, anti-CD34 and anti-CD14, then cells were fixed, permeabilized and stained with fluorochrome-conjugated anti-collagen I monoclonal antibodies. Results: Patients with IPF displayed almost undetectable levels of circulating fibrocytes, low levels of CEC, and normal levels of EPC. Patients treated with nintedanib displayed higher levels of CEC, but lower levels of endothelial cells expressing CD309 (the type II receptor for vascular endothelial growth factor). Treatment with both nintedanib and pirfenidone reduced the percentage of CEC and circulating fibrocytes. Conclusions: Levels of CEC were reduced in patients with IPF as compared to healthy individuals. The anti-fibrotic treatments ninted

Background: Chronic, progressive respiratory symptoms are associated with great psychological and emotional impact in patients suffering from interstitial lung disease (ILD). This single-centre pilot study evaluated for the first time the safety, feasibility and efficacy of a Mindfulness Based Stress Reduction Program (MBSR) in a group of patients with ILD. Methods: Prospective observational study set in a university hospital ILD outpatient clinic. Nineteen patients with different ILDs were recruited 2 months prior to the start of the 8-week MBSR program and followed up for 12 months. Primary outcomes were program safety and feasibility, while secondary outcomes were changes in moods and stress (assessed by Profile Of Mood State (POMS) and Perceived Stress Scale (PSS) questionnaires), symptoms (Shortness Of Breath (SOB) and Cough And Sputum Assessment (CASA-Q) questionnaires), lung function and exercise tolerance at 12 months. Results: Two patients (10.5%) dropped out in the observational period before the start of the MBSR intervention because of non-respiratory causes. All 17 patients who entered the 8-week MBSR program managed to complete it with an adherence average of eight sessions of nine. No adverse events related to the mindfulness training were reported. Statistically significant improvements in the POMS total score and in several individual items of POMS and PSS were observed throughout the study. However, respiratory questionnaire scores, lung function and exercise tolerance did not show a significant difference over time. Conclusions: An MBSR program appears to be safe and feasible in patients with ILD, and might affect perceived moods and stress producing a positive and lasting improvement in several stress-related negative domains. These findings pave the way to larger (possibly multicentre), randomised, controlled confirmatory trials.

Idiopathic interstitial pneumonias (IIPs) are a group of pulmonary disorders with distinct histologic and radiologic appearances and no identiiable cause. The new classiication of IIPs published in 2013 distinguishes six distinct major entities, including chronic, usually progressive ibrosing diseases, such as idiopathic pulmonary ibrosis (IPF) and idiopathic nonspeciic interstitial pneumonia. IPF, an invariably progressive and ultimately fatal lung disease that occurs in older adults, is the most frequent among the IIPs. Recent evidence and international guidelines advocate the importance of chest high-resolution computed tomography and multidisciplinary discussion (MDD) in the initial diagnostic assessment of patients with suspected IPF. MDD is currently considered the gold standard because improves the accuracy of IIPs diagnosis, avoiding unnecessary testing, and optimizing patient management, particularly nowadays that two drugs have been approved by regulatory agencies for the treatment of IPF. In this review, we focus on the revised diagnostic criteria for IIPs and IPF and provide an overview of the most recent clinical trials. Finally, we stress the fact that NSIP, one of the most frequent differential diagnosis in cases presenting with suspected IPF, is not anymore considered a provisional entity, but a deinite clinical-pathological entity.