Occurrence of idiopathic pulmonary fibrosis during immunosuppressive treatment: a case report

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

Use of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Case Study

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Mindfulness-based stress reduction in patients with interstitial lung diseases: a pilot, single-centre observational study on safety and efficacy

open11siBackground 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.openSgalla, Giacomo; Cerri, Stefania; Ferrari, Roberto; Ricchieri, Maria Pia; Poletti, Stefano; Ori, Margherita; Garuti, Martina; Montanari, Gloria; Luppi, Fabrizio; Petropulacos, Kyriakoula; Richeldi, LucaSgalla, Giacomo; Cerri, Stefania; Ferrari, Roberto; Ricchieri, Maria Pia; Poletti, Stefano; Ori, Margherita; Garuti, Martina; Montanari, Gloria; Luppi, Fabrizio; Petropulacos, Kyriakoula; Richeldi, Luc

COVID-19 Vaccine in Patients with Exacerbation of Idiopathic Pulmonary Fibrosis

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Levels of circulating endothelial cells are low in idiopathic pulmonary fibrosis and are further reduced by anti-fibrotic treatments

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 nintedanib and pirfenidone further reduced CEC levels. These findings might help explain the mechanism of action of these drugs and should be explored as predictive biomarkers in IPF

"Velcro-type" crackles predict specific radiologic features of fibrotic interstitial lung disease

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

Characterisation of lung sounds for early identification and monitoring of fibrotic interstitial lung disease

Chronic Interstitial Lung Diseases (ILD) are characterised by the interstitial involvement of the lungs, often resulting in the aberrant deposition of fibrotic tissue which causes progressive impairment of lung function. Earlier diagnosis of Idiopathic Pulmonary Fibrosis (IPF), the most frequent and severe among Idiopathic Interstitial Pneumonias, is warranted for a timely start of effective antifibrotic treatment. New tools for a more accurate prediction of disease progression and poor outcome are also required to improve the management of the individual patients. Audible “Velcro-type” crackles on chest auscultation represent a typical finding in patients with fibrotic ILD. Although non-specific, their assessment performed by healthcare practitioners is invaluable to prompt the diagnostic work up and limit the diagnostic delay in these patients. Nevertheless, their value toward the early identification and the monitoring of fibrotic ILD has never been explored. Despite the modern advances in the field of electronic auscultation and computerised analysis of lung sounds offer a reliable, objective characterisation of normal and adventitious lung sounds, such methods have never been successfully translated into clinical practice. This thesis provides a comprehensive characterisation of lung sounds recorded in patients with fibrotic ILD using a digital stethoscope, and tries to clarify whether this approach may valuably inform the diagnostic process and the management of these patients in the clinical practice. A prospective case-control study investigated the association between the presence of “Velcrotype” crackles, evaluated by respiratory physicians, and radiologic signs of pulmonary fibrosis in three consecutive large cohorts of patients undergoing a High-Resolution Computerised Tomography (HRCT) scan of the chest. The presence of “Velcro-type” crackles predicted the presence of ILD patterns on HRCT and was independently associated with distinct radiologic features suggestive of pulmonary fibrosis. Such evidence indicates that lung sounds have potential to improve early identification of ILD, while future work is needed to determine the real diagnostic accuracy of “Velcro-type” crackles, either assessed subjectively or via quantitative computerised analysis. A longitudinal cohort study collected serial recordings of lung sounds from a cohort of 19 patients with IPF and 10 healthy controls over a 12-month observation period. Computer-aided lung sound analysis was used to extract almost 500 different acoustic features from each recording. A narrow set of acoustic features of lung sounds recorded via a digital stethoscope was found to be highly reproducible, distinctive of pulmonary fibrosis and responsive to disease progression assessed via validated clinical and functional parameters. As such, this study provided the first evidence on the longitudinal changes occurring in the acoustic features of lung sounds from IPF patients, and paves the way to further research aimed to determine the validity of lung sounds as a new prognostic marker in patients with progressive ILD

Long-term management of IPF with pirfenidone - A clinical case study with 5 years follow-up

Idiopathic pulmonary fibrosis (IPF) is a progressively fibrotic interstitial lung disease that is associated with a median survival of 2-5 years from initial diagnosis.To date, the search for an effective treatment has involved numerous clinical trials of investigational agents but without significant success. Nevertheless, research over the past 10 years has provided us with a wealth of information on its histopathology, diagnostic work-up, and a greater understanding of its pathophysiology. Specifically, IPF is no longer thought to be a predominantly pro-inflammatory disorder. Rather, the fibrosis in IPF is increasingly understood to be the result of a fibroproliferative and aberrant wound healing cascade. The development of therapeutic targets has therefore shifted in accordance with this paradigm change. Emerging clinical data from recently published and ongoing trials investigating new potential pharmacological agents should be considered in the routine clinical management of these patients. Based upon encouraging results from randomised-controlled trials showing a positive effect in slowing decline in pulmonary function and reducing disease progression, pirfenidone was approved in 2011 as the first treatment in patients with IPF. This case study describes the clinical course of a patient enrolled into the Phase III and open-label extension studies of pirfenidone