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Machine Learning (ML) is an algorithm based on big data, which learns patterns from the previously observed data through classifying, predicting, and optimizing to accomplish specific tasks. In recent years, there has been rapid development in the field of ML in medicine, including lung imaging analysis, intensive medical monitoring, mechanical ventilation, and there is need for intubation etiology prediction evaluation, pulmonary function evaluation and prediction, obstructive sleep apnea, such as biological information monitoring and so on. ML can have good performance and is a great potential tool, especially in the imaging diagnosis of interstitial lung disease. Idiopathic pulmonary fibrosis (IPF) is a major problem in the treatment of respiratory diseases, due to the abnormal proliferation of fibroblasts, leading to lung tissue destruction. The diagnosis mainly depends on the early detection of imaging and early treatment, which can effectively prolong the life of patients. If the computer can be used to assist the examination results related to the effects of fibrosis, a timely diagnosis of such diseases will be of great value to both doctors and patients. We also previously proposed a machine learning algorithm model that can play a good clinical guiding role in early imaging prediction of idiopathic pulmonary fibrosis. At present, AI and machine learning have great potential and ability to transform many aspects of respiratory medicine and are the focus and hotspot of research. AI needs to become an invisible, seamless, and impartial auxiliary tool to help patients and doctors make better decisions in an efficient, effective, and acceptable way. The purpose of this paper is to review the current application of machine learning in various aspects of respiratory diseases, with the hope to provide some help and guidance for clinicians when applying algorithm models. [ABSTRACT FROM AUTHOR]

Introduction: Telomeropathies are associated with a wide range of diseases and less common combinations of various pulmonary and extrapulmonary disorders. Case presentation: In proband with high-risk myelodysplastic syndrome and interstitial pulmonary fibrosis, whole exome sequencing revealed a germline heterozygous variant of CTC1 gene (c.1360delG). This "frameshift" variant results in a premature stop codon and is classified as likely pathogenic/pathogenic. So far, this gene variant has been described in a heterozygous state in adult patients with hematological diseases such as idiopathic aplastic anemia or paroxysmal nocturnal hemoglobinuria, but also in interstitial pulmonary fibrosis. Described CTC1 gene variant affects telomere length and leads to telomeropathies. Conclusions: In our case report, we describe a rare case of coincidence of pulmonary fibrosis and hematological malignancy caused by a germline gene mutation in CTC1. Lung diseases and hematologic malignancies associated with short telomeres do not respond well to standard treatment. [ABSTRACT FROM AUTHOR]

The current study aimed to evaluate rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) in clinical practice and whether disease characteristics are associated with X-ray and high-resolution computed tomography (HR-CT) findings. Medical history of RA patients from a tertiary rheumatology clinic was retrieved from its electronic database starting from 1 January 2019 until the study date (8 August 2022) using International Classification of Disease version 10 codes for RA, ILD and exclusion criteria. The study included 78 RA patients (75.6% women, 15.4% active smokers), with average time from RA to ILD of 5.6 years. Regarding chest X-ray findings, men had a higher prevalence of nodules, combined fibrosis and nodules and combined bronchiectasis and nodules, rheumatoid factor (RF)-positive patients had a higher prevalence of fibrosis and anti-cyclic citrullinated peptide antibodies (ACPA)-positive patients had a higher prevalence of bronchiectasis. Regarding HR-CT findings, patients actively treated with methotrexate had a higher prevalence of nodules; a combination of fibrosis and nodules; combination of emphysema and nodules; and combination of fibrosis, emphysema and nodules. ILD develops within approximately 5 years from RA diagnosis, and ILD-associated imaging findings on chest X-rays and HR-CT are more prevalent among men with RA, among patients with positive RA serology (RF and/or ACPA) and RA patients on methotrexate. [ABSTRACT FROM AUTHOR]

Introduction: Systemic sclerosis (SSc) is an autoimmune disorder that causes vasculopathy and scarring, most commonly in the lungs and skin, but it can also affect other organs. Endothelial vinculin plays a critical role in angiogenesis regulation. Therefore, vinculin overexpression in SSc may give rise to anti-vinculin antibodies, which may contribute to the development of SSc vasculopathy. The current research aims to (1) determine whether anti-vinculin autoantibodies play a significant role in the diagnosis of SSc and (2) compare anti-vinculin serum levels between two scleroderma patient populations, namely, pulmonary artery hypertension (PAH)–predominant and interstitial pulmonary fibrosis (IPF)–predominant groups. Methods: This research included 140 participants categorized into three groups: group I—patients with PAH-predominant; group II—patients with ILD-predominant; group III—the control group. Anti-vinculin antibodies were detected in serum samples collected from all participants using ELISA. All subjects underwent high-resolution computed tomography (CT), diffusing capacity for carbon monoxide, and pulmonary function tests. Results: Patients in group I (PAH-predominant group, N = 35) were 41.3 [± 11.4] years old, with 80% being women. Patients in group II (ILD-predominant group, N = 35) were 41.0 [± 11.5] years old. The SSc group showed significantly higher anti-vinculin antibody levels than the control group (P < 0.001). The PAH-predominant group demonstrated significantly higher anti-vinculin antibody levels and anti-vinculin positivity than the ILD-predominant group. Conclusion: Anti-vinculin antibodies in the blood appear to be diagnostic biomarkers for scleroderma. Furthermore, they shed light on some novel perspectives on the pathophysiology of specific lung fibrotic changes. Key Points • This study included two groups of systemic sclerosis patients (PAH-predominant group, ILD-predominant group) as well as a control group to investigate the significance of anti-vinculin antibodies in such cases. • Our results have demonstrated that anti-vinculin antibodies can play a significant role in diagnosing and monitoring systemic sclerosis disease. [ABSTRACT FROM AUTHOR]

Background Idiopathic pulmonary fibrosis (IPF) is the most common form of interstitial lung disease. Anemia is a common finding in patients with IPF. The role of erythropoietin (EPO) in IPF-related anemia is rarely discussed. The present study aimed to determine EPO levels in patients with IPF and its relation to various clinical parameters. Patients and methods This prospective study was conducted on 45 patients with IPF and 45 healthy nonanemic controls. Included patients were subjected to careful history taking, thorough clinical examination, and pulmonary function testing. Investigations included C-reactive protein (CRP), serum electrolytes, blood gases, and serum EPO. Results The present study included 45 patients with IPF. They comprised 31 (68.9%) females and 14 (31.1%) males. Among the studied patients, there were 17 anemic patients. Anemic patients had significantly lower forced expiratory volume first second, higher erythrocyte sedimentation rate, and higher CRP levels. Patients have significantly higher EPO levels when compared with controls. There was a significant inverse correlation between EPO and forced expiratory volume first second in all patients. In addition, there was a significant direct correlation between EPO and CRP levels. Conclusion Anemia is prevalent in patients with IPF. In those patients, EPO levels are elevated but EPO response remains poor. EPO levels are directly correlated with CRP levels.

Machine Learning (ML) is an algorithm based on big data, which learns patterns from the previously observed data through classifying, predicting, and optimizing to accomplish specific tasks. In recent years, there has been rapid development in the field of ML in medicine, including lung imaging analysis, intensive medical monitoring, mechanical ventilation, and there is need for intubation etiology prediction evaluation, pulmonary function evaluation and prediction, obstructive sleep apnea, such as biological information monitoring and so on. ML can have good performance and is a great potential tool, especially in the imaging diagnosis of interstitial lung disease. Idiopathic pulmonary fibrosis (IPF) is a major problem in the treatment of respiratory diseases, due to the abnormal proliferation of fibroblasts, leading to lung tissue destruction. The diagnosis mainly depends on the early detection of imaging and early treatment, which can effectively prolong the life of patients. If the computer can be used to assist the examination results related to the effects of fibrosis, a timely diagnosis of such diseases will be of great value to both doctors and patients. We also previously proposed a machine learning algorithm model that can play a good clinical guiding role in early imaging prediction of idiopathic pulmonary fibrosis. At present, AI and machine learning have great potential and ability to transform many aspects of respiratory medicine and are the focus and hotspot of research. AI needs to become an invisible, seamless, and impartial auxiliary tool to help patients and doctors make better decisions in an efficient, effective, and acceptable way. The purpose of this paper is to review the current application of machine learning in various aspects of respiratory diseases, with the hope to provide some help and guidance for clinicians when applying algorithm models.

Abstract Background Genetic testing is proposed for suspected cases of monogenic pulmonary fibrosis, but clinicians and patients need specific information and recommendation about the related diagnosis and management issues. Because multidisciplinary discussion (MDD) has been shown to improve accuracy of interstitial lung disease (ILD) diagnosis, we evaluated the feasibility of a genetic MDD (geneMDD) dedicated to the indication for and interpretation of genetic testing. The geneMDD group met monthly and included pediatric and adult lung specialists with ILD expertise, molecular and clinical geneticists, and one radiologist. Hematologists, rheumatologists, dermatologists, hepatologists, and pathologists were also invited to attend. Results Since 2016, physicians from 34 different centers in 7 countries have participated in the geneMDD. The medical files of 95 patients (53 males) have been discussed. The median age of patients was 43 years [range 0–77], 10 were ≤ 15 years old, and 6 were deceased at the time of the discussion. Among 85 analyses available, the geneMDD considered the rare gene variants pathogenic for 61: 37 variants in telomere-related genes, 23 variants in surfactant-related genes and 1 variant in MARS. Genetic counseling was offered for relatives of these patients. The geneMDD therapeutic proposals were as follows: antifibrotic drugs (n = 25), steroids or immunomodulatory therapy (n = 18), organ transplantation (n = 21), watch and wait (n = 21), or best supportive care (n = 4). Conclusion Our experience shows that a dedicated geneMDD is feasible regardless of a patient’s age and provides a unique opportunity to adapt patient management and therapy in this very rare condition.

The current study aimed to evaluate rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) in clinical practice and whether disease characteristics are associated with X-ray and high-resolution computed tomography (HR-CT) findings. Medical history of RA patients from a tertiary rheumatology clinic was retrieved from its electronic database starting from 1 January 2019 until the study date (8 August 2022) using International Classification of Disease version 10 codes for RA, ILD and exclusion criteria. The study included 78 RA patients (75.6% women, 15.4% active smokers), with average time from RA to ILD of 5.6 years. Regarding chest X-ray findings, men had a higher prevalence of nodules, combined fibrosis and nodules and combined bronchiectasis and nodules, rheumatoid factor (RF)-positive patients had a higher prevalence of fibrosis and anti-cyclic citrullinated peptide antibodies (ACPA)-positive patients had a higher prevalence of bronchiectasis. Regarding HR-CT findings, patients actively treated with methotrexate had a higher prevalence of nodules; a combination of fibrosis and nodules; combination of emphysema and nodules; and combination of fibrosis, emphysema and nodules. ILD develops within approximately 5 years from RA diagnosis, and ILD-associated imaging findings on chest X-rays and HR-CT are more prevalent among men with RA, among patients with positive RA serology (RF and/or ACPA) and RA patients on methotrexate.

The coronavirus disease 2019 (COVID-19) pandemic has caused considerable socio-economic burden, which fueled the development of treatment strategies and vaccines at an unprecedented speed. However, our knowledge on disease recovery is sparse and concerns about long-term pulmonary impairments are increasing. Causing a broad spectrum of symptoms, COVID-19 can manifest as acute respiratory distress syndrome (ARDS) in the most severely affected patients. Notably, pulmonary infection with Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2), the causing agent of COVID-19, induces diffuse alveolar damage (DAD) followed by fibrotic remodeling and persistent reduced oxygenation in some patients. It is currently not known whether tissue scaring fully resolves or progresses to interstitial pulmonary fibrosis. The most aggressive form of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF). IPF is a fatal disease that progressively destroys alveolar architecture by uncontrolled fibroblast proliferation and the deposition of collagen and extracellular matrix (ECM) proteins. It is assumed that micro-injuries to the alveolar epithelium may be induced by inhalation of micro-particles, pathophysiological mechanical stress or viral infections, which can result in abnormal wound healing response. However, the exact underlying causes and molecular mechanisms of lung fibrosis are poorly understood due to the limited availability of clinically relevant models. Recently, the emergence of SARS-CoV-2 with the urgent need to investigate its pathogenesis and address drug options, has led to the broad application of in vivo and in vitro models to study lung diseases. In particular, advanced in vitro models including precision-cut lung slices (PCLS), lung organoids, 3D in vitro tissues and lung-on-chip (LOC) models have been successfully employed for drug screens. In order to gain a deeper understanding of SARS-CoV-2 infection and ultimately alveolar tissue regeneration, it will be crucial to optimize the available models for SARS-CoV-2 infection in multicellular systems that recapitulate tissue regeneration and fibrotic remodeling. Current evidence for SARS-CoV-2 mediated pulmonary fibrosis and a selection of classical and novel lung models will be discussed in this review.

Ozone is known to cause bronchial inflammation and airway hyper-responsiveness via oxidative injury and inflammation. While other ambient air pollutants such as particulate matter (PM) and nitrogen dioxide showed decreasing trends in mean annual concentrations, ozone concentrations have not declined recently in most countries across the world. Short-term exposure to high concentrations of ozone has been associated with increased mortality and cardiovascular and respiratory morbidity in many regions of the world. However, the long-term effects of ozone have been less investigated than the short-term exposure due to the difficulty in modeling ozone exposure and linking between individual exposures and health outcome data. A recently developed model of ozone exposure enabled the investigation of long-term ozone effects on health outcomes. Recent findings suggested that long-term exposure to ozone was associated with an increased risk of cardiovascular and respiratory mortality. Longitudinal studies using large cohorts also revealed that long-term exposure to ozone was associated with a greater decline in lung function and the progression of emphysema. The development of long-term standards for ozone as well as PM should be considered to protect the respiratory health of the general population and people with chronic respiratory diseases.

People worldwide are living longer, and it is estimated that by 2050, the proportion of the world's population over 60 years of age will nearly double. Natural lung aging is associated with molecular and physiological changes that cause alterations in lung function, diminished pulmonary remodeling and regenerative capacity, and increased susceptibility to acute and chronic lung diseases. As the aging population rapidly grows, it is essential to examine how alterations in cellular function and cell-to-cell interactions of pulmonary resident cells and systemic immune cells contribute to a higher risk of increased susceptibility to infection and development of chronic diseases, such as chronic obstructive pulmonary disease and interstitial pulmonary fibrosis. This review provides an overview of physiological, structural, and cellular changes in the aging lung and immune system that facilitate the development and progression of disease. [ABSTRACT FROM AUTHOR]

Abstract Introduction Health-related quality-of-life questionnaires need to be incorporated into the evaluations of idiopathic pulmonary fibrosis (IPF) patients to assess their influence. Aim The aim of the study was to evaluate the validity of generic and specific questionnaires in assessing the severity of IPF in correlation with high-resolution computed tomography (HRCT), diffusion lung capacity for carbon monoxide (DLCO), and cardiopulmonary exercise testing (CPET). Patients and methods Forty stable IPF patients were prospectively recruited and categorized on the basis of spirometry, DLCO, HRCT, and CPET. The results were correlated with a generic International Physical Activity Questionnaire (IPAQ) and a Specific Saint George Respiratory Questionnaire (SGRQ). Results IPF patients showed restrictive pattern with impairment of diffusion capacity (forced vital capacity (FVC)= 56±14.8% and DLCO=48.5±20% of predicted value) with a total semiquantitative scoring of HRCT 16.6±8. The mean total score of the SGRQ questionnaire for all studied cases was 56.5+21 and categorical scoring of IPAQ showed that 45, 42.5, and 12.5% of patients were in moderate, severe, and mild categories, respectively. There was a negative correlation between the total score of SGRQ and VO2max (ml/kg/min) (maximum oxygen consumption) (r=−0.35) and VE’ (l/min) (minute ventilation) (r=−0.39) on CPET, as well as with DLCO (r=−0.53), and a positive correlation with HRCT score (r=0.63). There was a highly significant correlation between IPAQ and VO2max (χ2=28), VE’ (χ2=14.8) and desaturation percentage variables of CPET, DLCO (r=0.61), and HRCT score (r=−0.68). Conclusion Correlations between physiological parameters including DLCO and CPET, radiological parameters in the form of HRCT, and health-related quality-of-life assessment using SGRQ and IPAQ were strong and it was possible to distinguish IPF patients with severely impaired lung functions.

Aim of work: Idiopathic pulmonary fibrosis (IPF) is the second most common cause of admission to Chest Department at Assiut University hospital, the pattern of presentation, method for diagnosis and Co-morbidities need further studies. Aim: To explore demographic data and pattern of presentation of patients with IPF in upper Egypt and to study the difference from international data. Methods: A total of 568 patients with final diagnosis of IPF were studied, retrospective study was done using the available hospital database for all patients admitted at Assiut University hospital (Tertiary hospital for all upper Egypt Governorates) during the period from 2007 to 2012. Patients with incomplete data were excluded from study, all patients underwent chest X-ray, high resolution computed tomography, spirometry, arterial blood gases, in addition to routine laboratory investigation. Patients with clinical or laboratory evidence of collagen vascular disease or extrinsic allergic alveolitis were excluded from current study. Results: The current study included 568 patients, 191 males and 377 females, mean age 44 ± 12 years. In all cases diagnosis was made according to clinical, spirometry and HRCT chest, no one was subjected to thoracoscopic or bronchoscopic lung biopsy. Most cases were house wife or farmer, 76% of cases were non-smokers, 17% ex-smokers and 7% current smokers, 86% of cases have restrictive spirometry. Usual interstitial pneumonia was the most common high resolution chest computed tomography pattern (51%) (Fig. 5). 39% of cases have at least one Co-morbid disease such as systemic hypertension, ischemic heart disease, pulmonary embolism and/or diabetes mellitus. There was a significant difference between male and female patients with IPF with regard to smoking status (P value

Abstract Mitochondrial reactive oxygen species production may lead to tissue injury associated with two respiratory disorders of unknown origin which are shared by common tissue fibrosis, IPF and sarcoidosis. Sequence analysis of 22 mt-tRNA genes and parts of their flanking genes revealed 32 and 45 mutations in 38/40 IPF and 69/85 sarcoidosis patients respectively. 4 novel mutations were identified. 15/32 and 25/45 mutations were exclusively expressed while 12/32 and 17/45 mutations predominantly occurred in IPF and sarcoidosis group respectively, compared to healthy controls. Novel mutation combinations were solely expressed in disease. Hence, a mitochondrial-mediated pathogenic pathway seems to underlie both entities. Highlights • A mitochondrial-mediated pathogenic pathway seems to underlie IPF. • Mitochondrial alterations and redox imbalance seem to underlie sarcoidosis. • Previously unrecognized defects in mt-tRNAs and their flanking genes presented in IPF. • Novel defects in mt-tRNAs and their flanking genes occurred in sarcoidosis. [ABSTRACT FROM AUTHOR]

Ultrasound (US) is increasing its potential in the assessment of several rheumatic disorders. Recently, different applications of this imaging technique have emerged. Interesting data supporting its utility and validity in the assessment of the lung to detect and quantify interstitial pulmonary fibrosis in rheumatic diseases, even in subclinical phases, have been reported. The main purpose of this review is to provide an overview of the role of US in the assessment of interstitial pulmonary fibrosis in rheumatic disorders and to discuss the current evidence supporting its clinical relevance in daily clinical practice. [ABSTRACT FROM AUTHOR]

Background To determine whether the HRCT findings are useful to differentiate asbestosis from idiopathic pulmonary fibrosis (IPF). Methods We assessed HRCT scans from patients with asbestosis (n = 96) and IPF (n = 65). The frequencies and extent of parenchymal abnormalities and the frequencies of pleural changes were evaluated by consensus of two chest radiologists. Results There was a significant difference between IPF and asbestosis in pleural changes. In addition, there were significant differences between IPF and asbestosis in several parenchymal abnormalities on CT, especially in the less advanced stage of both diseases. On multivariate analysis, HRCT features that distinguished asbestosis from IPF were subpleural lines at a distance of less than 5 mm from the inner chest wall, subpleural dots and parenchymal bands. Conclusions There are significant differences between IPF and asbestosis in the parenchymal and pleural abnormalities on CT. Am. J. Ind. Med. 59:301-306, 2016. © 2016 Wiley Periodicals, Inc. [ABSTRACT FROM AUTHOR]

Objective. MCTD is a chronic, immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features, including progressive lung fibrosis. The aim of the study was to evaluate the potential impact of anti-SSA (i.e. Ro52 and Ro60) and anti-SSB autoantibodies as markers for disease outcomes in MCTD. Methods. Stored serum samples from 113 patients included in the cross-sectional, nationwide Norwegian MCTD cohort were screened for the presence of anti-Ro52, anti-Ro60 and anti-SSB by a commercial line immunoassay. Correlation analyses were carried out with clinical parameters, including quantitative lung fibrosis scores by high-resolution CT. Lung fibrosis was defined by reticular pattern changes according to the Fleischner Society CT criteria for interstitial lung disease. Results. Anti-Ro52 antibodies were present in 29%, anti-Ro60 in 19% and anti-SSB in 6% of the MCTD sera. High-resolution CT scoring identified lung fibrosis in 38 of 113 (34%) MCTD patients. Anti-Ro52 antibodies were detected in 50% (19 of 38) of the MCTD patients with lung fibrosis and in 19% (14 of 75) without lung fibrosis (P<0.001). The odds ratio for the presence of anti-Ro52 antibodies in lung fibrosis was 4.4 (95% CI 1.8, 10.3). Anti-Ro52 antibodies were equally frequent in patients with mild to moderate (eight of 17; 44%) and severe fibrosis (11 of 21; 52%). Anti-Ro52 was not associated with any of the other clinical parameters assessed, nor was anti-Ro60 or anti-SSB. Conclusion. Our cross-sectional data suggest that anti-Ro52 antibodies may serve as a potential marker for lung fibrosis in MCTD. [ABSTRACT FROM AUTHOR]

Os autores descrevem o caso de uma paciente com esclerose sistêmica (ES) - forma limitada - com comprometimento pulmonar tipo fibrose intersticial. Após sete anos sem acompanhamento, foram identificados gânglios mediastinais e esplenomegalia. A biópsia de linfonodos mostrou granuloma não caseoso sugestivo de sarcoidose. Estamos mostrando, neste caso, a associação de ES e sarcoidose, para chamar a atenção para esse fato e enfatizar que a sarcoidose deve ser lembrada no diagnóstico diferencial das complicações pulmonares da esclerodermia.The authors describe the case of a patient with limited scleroderma and interstitial lung disease. Follow-up was lost for seven years, when patient returned presenting nodular mediastinal enlargement and splenomegaly. Lymph node biopsy showed granulomatous lesions without caseum suggestive of sarcoidosis. This case is being presented to remind the association of scleroderma and sarcoidosis as a possible differential diagnosis of scleroderma pulmonary complications.

Aim. To ascertain the role of lung biopsy in diagnosis of lung lesions in hemoblastosis (HB) patients. Material and methods. The results of diagnostic biopsies of the lungs obtained from 22 HВ patients are presented. Ten patients had no respiratory insufficiency (RI), twelve patients had RI. The biopsy was transbronchial in 1 case, thoracoscopic in 10 and open in 11 cases. Results. In Rl-free patients lung biopsy was informative in all the cases. The biopsy provided information which allowed therapy modification resulting in improvement of the patient condition. In RI patients biopsy was informative in 8 of 12 patients. Nonspecific changes in the lungs were identified histologically in 2 of 12 patients. In 2 RI patients lung biopsy confirmed the diagnosis made after examination of the bronchoalveolar lavage. Modification of therapy after the biopsy was conducted in 58.3% HB patients with RI. Improvement was seen in 2 of them. 10 of 12 patients with RI died within 1-2 weeks after biopsy. Conclusion. Lung biopsy in HB patients should be obtained only after examination with noninvasive methods and before development of RI as prognosis after lung biopsy in the presence of RI is unfa- vourable. The histological material should be examined for all expected pathogens.

Objective To describe clinical canine patients with naturally occurring pulmonary hypertension and radiographic pulmonary alveolar infiltrates before and after treatment with sildenafil. Animals Ten client-owned dogs. Methods A retrospective analysis of dogs with echocardiographically-determined pulmonary hypertension and pulmonary alveolar infiltrates on thoracic radiographs was performed before (PRE) and after (POST) sildenafil therapy. Clinical scores, pulmonary alveolar infiltrate scores and tricuspid regurgitation gradients were analyzed PRE and POST sildenafil. Results Pulmonary alveolar infiltrates associated with pulmonary hypertension developed in a diffusely patchy distribution (10/10). Sixty percent of dogs had a suspected diagnosis of interstitial pulmonary fibrosis as the etiology of pulmonary hypertension. Median PRE clinical score was 4 (range: 3–4) compared to POST score of 0 (0–2) (p = 0.005). Median alveolar infiltrate score PRE was 10 (5–12) compared to POST score of 4 (0–6) (p = 0.006). Median tricuspid regurgitation gradient PRE was 83 mmHg (57–196) compared to 55 mmHg POST (33–151) (p = 0.002). Conclusions A subset of dogs with moderate to severe pulmonary hypertension present with diffuse, patchy alveolar infiltrates consistent with non-cardiogenic pulmonary edema. The typical clinical presentation is acute dyspnea and syncope, often in conjunction with heart murmurs suggestive of valvular insufficiency. This constellation of signs may lead to an initial misdiagnosis of congestive heart failure or pneumonia; however, these dogs clinically and radiographically improve with the initiation of sildenafil. [ABSTRACT FROM AUTHOR]

Introduction. Cryptogenic organizing pneumonia is a particular form of inflammatory and fibroproliferative lung disease. The disease onset is subacute with cough, dyspnoea, fever, weight loss, and elevation of biological inflammatory markers. Chest imaging usually shows multifocal alveolar opacities predominating in the subpleural regions. Lung biopsy reveals budding connective tissue filling the distal airspaces. Case outline. A 57-year-old electrician complaining of cough, dyspnoea, and fatigue was diagnosed with pneumonia and treated with antibiotics and antihistaminics. After clinical and radiographic progression of the disease, open lung biopsy was performed, some 15 months after the disease onset. The diagnosis of cryptogenic organising pneumonia was made. The patient was treated with oral and inhalatory corticosteroids and finally with cytostatics, which led to a partial improvement of his condition. However, work capacity was lost and the quality of life seriously deteriorated. Conclusion. The diagnosis is established by combining clinical, radiological and histological criteria. Similarities with other disease processes can lead to a delayed or erroneous diagnosis. Most patients respond well to corticosteroid therapy (prednisone or methyl-prednisolone). Relapses are frequent but can generally be controlled.

The current study aimed to evaluate rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) in clinical practice and whether disease characteristics are associated with X-ray and high-resolution computed tomography (HR-CT) findings. Medical history of RA patients from a tertiary rheumatology clinic was retrieved from its electronic database starting from 1 January 2019 until the study date (8 August 2022) using International Classification of Disease version 10 codes for RA, ILD and exclusion criteria. The study included 78 RA patients (75.6% women, 15.4% active smokers), with average time from RA to ILD of 5.6 years. Regarding chest X-ray findings, men had a higher prevalence of nodules, combined fibrosis and nodules and combined bronchiectasis and nodules, rheumatoid factor (RF)-positive patients had a higher prevalence of fibrosis and anti-cyclic citrullinated peptide antibodies (ACPA)-positive patients had a higher prevalence of bronchiectasis. Regarding HR-CT findings, patients actively treated with methotrexate had a higher prevalence of nodules; a combination of fibrosis and nodules; combination of emphysema and nodules; and combination of fibrosis, emphysema and nodules. ILD develops within approximately 5 years from RA diagnosis, and ILD-associated imaging findings on chest X-rays and HR-CT are more prevalent among men with RA, among patients with positive RA serology (RF and/or ACPA) and RA patients on methotrexate.

BACKGROUND: Admitting patients with interstitial lung disease (ILD) to the ICU is controversial, due to their associated high mortality when they require invasive mechanical ventilation. We aimed to determine the risk factors for mortality in ILD patients requiring ICU support due to acute respiratory failure. METHODS: An observational cohort study was performed in 2 chest diseases teaching hospitals. We included all ILD patients with acute respiratory failure admitted between 2008 and 2010. Subject demographics, noninvasive ventilation (NIV) and invasive ventilation use, and mortality were obtained from medical records. Subjects receiving NIV were divided based on their continuous or non-continuous demand for NIV. NIV failure was defined as intubation for invasive ventilation, or death during NIV. Cox regression analysis was used to determine the hazard ratio for NIV failure. RESULTS: We enrolled 120 subjects: 71 male, median age 66 years. The types of ILD were idiopathic pulmonary fibrosis (n = 96), collagen vascular disease (n = 10), silicosis (n = 9), drug induced (n = 3), and eosinophilic pneumonia (n = 2). The median (IQR) Acute Physiology and Chronic Health Evaluation (APACHE II) score was 24 (19 -31), and 75 (62.5%) subjects received NIV on ICU admission, 47 (62.7%) of whom needed continuous NIV. The NIV failure rate was 49.3% (n = 37). The mortality rates of continuous NIV, non-continuous NIV, invasive ventilation, and total ICU were 61.7% (29/47), 10.7% (3/28), 89.7% (61/68), 60% (72/120), respectively. APACHE II > 20 and continuous NIV demand indicated significant risk for NIV failure: hazard ratio 2.77 (95% CI 1.19-6.45), P < .02, and 5.12, (1.44 -18.19), P < .01, respectively. CONCLUSIONS: Because of higher mortality, physicians should consider invasive ventilation cautiously in the ICU management of ILD patients with acute respiratory failure. NIV may be an option in less severely ill patients with APACHE II score < 20. [ABSTRACT FROM AUTHOR]

Lung vascular alterations and pulmonary hypertension associated with oxidative stress have been reported to be involved in idiopathic lung fibrosis (ILF). Therefore, here, we hypothesize that the widely used lung fibrosis inducer, bleomycin, would cause cytoskeletal rearrangement through thiol-redox alterations in the cultured lung vascular endothelial cell (EC) monolayers. We exposed the monolayers of primary bovine pulmonary artery ECs to bleomycin (10 µg) and studied the cytotoxicity, cytoskeletal rearrangements, and the macromolecule (fluorescein isothiocyanate-dextran, 70,000 mol. wt.) paracellular transport in the absence and presence of two thiol-redox protectants, the classic water-soluble N-acetyl- l-cysteine (NAC) and the novel hydrophobic N, N′-bis-2-mercaptoethyl isophthalamide (NBMI). Our results revealed that bleomycin induced cytotoxicity (lactate dehydrogenase leak), morphological alterations (rounding of cells and filipodia formation), and cytoskeletal rearrangement (actin stress fiber formation and alterations of tight junction proteins, ZO-1 and occludin) in a dose-dependent fashion. Furthermore, our study demonstrated the formation of reactive oxygen species, loss of thiols (glutathione, GSH), EC barrier dysfunction (decrease of transendothelial electrical resistance), and enhanced paracellular transport (leak) of macromolecules. The observed bleomycin-induced EC alterations were attenuated by both NAC and NBMI, revealing that the novel hydrophobic thiol-protectant, NBMI, was more effective at µM concentrations as compared to the water-soluble NAC that was effective at mM concentrations in offering protection against the bleomycin-induced EC alterations. Overall, the results of the current study suggested the central role of thiol-redox in vascular EC dysfunction associated with ILF. [ABSTRACT FROM AUTHOR]

The mechanisms of lung microvascular complications and pulmonary hypertension known to be associated with idiopathic pulmonary fibrosis (IPF), a debilitating lung disease, are not known. Therefore, we investigated whether bleomycin, the widely used experimental IPF inducer, would be capable of activating phospholipase D (PLD) and generating the bioactive lipid signal-mediator phosphatidic acid (PA) in our established bovine lung microvascular endothelial cell (BLMVEC) model. Our results revealed that bleomycin induced the activation of PLD and generation of PA in a dose-dependent (5, 10, and 100 µg) and time-dependent (2-12 hours) fashion that were significantly attenuated by the PLD-specific inhibitor, 5-fluoro-2-indolyl deschlorohalopemide (FIPI). PLD activation and PA generation induced by bleomycin (5 µg) were significantly attenuated by the thiol protectant (N-acetyl-L-cysteine), antioxidants, and iron chelators suggesting the role of reactive oxygen species (ROS), lipid peroxidation, and iron therein. Furthermore, our study demonstrated the formation of ROS and loss of glutathione (GSH) in cells following bleomycin treatment, confirming oxidative stress as a key player in the bleomycin-induced PLD activation and PA generation in ECs. More noticeably, PLD activation and PA generation were observed to happen upstream of bleomycin-induced cytotoxicity in BLMVECs, which was protected by FIPI. This was also supported by our current findings that exposure of cells to exogenous PA led to internalization of PA and cytotoxicity in BLMVECs. For the first time, this study revealed novel mechanism of the bleomycin-induced redox-sensitive activation of PLD that led to the generation of PA, which was capable of inducing lung EC cytotoxicity, thus suggesting possible bioactive lipid-signaling mechanism/mechanisms of microvascular disorders encountered in IPF. [ABSTRACT FROM PUBLISHER]

Abstract: Background and study aims: Several extrahepatic manifestations (EHMs) have been reported in the natural history of hepatitis C virus (HCV) infection. Some studies had demonstrated a higher frequency of HCV in patients with idiopathic interstitial pulmonary fibrosis (IPF). The aim of this study is to investigate interstitial pulmonary changes via high-resolution computed tomography (HRCT) and pulmonary function tests (PFTs) in patients with HCV infection. Patients and methods: Forty patients with HCV infection without diagnosis of any pulmonary diseases (group I) and 10 healthy persons (group II) were enrolled in the study. PFT and HRCT were performed in all cases. Results: Findings of interstitial pulmonary involvement were detected in the HRCT of 19 out of 40 patients (47.5%). A decrease lower than 80% of the predicted value was detected in forced vital capacity (FVC) with restrictive pattern together with post-exercise hypoxia, in 21 (52.5%) patients. There was a significant difference between both groups in the findings of HRCT (x 2 =7.66, df =1, p =0.004) and PFTs (x 2 =9.05, df =3, p =0.002). According to the Child–Pugh classification, in group I, 15 patients were of class A, 16 were of class B and nine of class C, with no significant difference between classes as regards HRCT findings and PFT values. Six patients were positive for serum cryoglobulins. In these patients, all the parameters were not related to age. Conclusion: Pulmonary interstitial changes are prevalent in HCV-infected patients, irrespective of the severity of their liver condition. It may also occur without respiratory symptoms. [Copyright &y& Elsevier]

Summary: Background: HRQL in IPF patients is impaired. Data from other respiratory diseases led us to hypothesize that significant gender differences in HRQL in IPF also exist. Methods: Data were drawn from the NIH-sponsored Lung Tissue Research Consortium (LTRC). Demographic and pulmonary physiology data along with MMRC, SF-12, and SGRQ scores from women vs. men were compared with two-sample t-tests. Multivariate linear regression was used to examine the association between SF-12 component scores and gender while adjusting for other relevant variables. Results: The study sample consisted of 147 men and 74 women. Among several baseline variables, only DLCO% predicted differed between women and men, (43.7 vs. 38.0, p =0.03). In general, men exhibited lower (better) MMRC scores (1.7 vs. 2.4, p =0.02), particularly those with milder disease as measured by DLCO% predicted. In an adjusted analysis, SF-12 PCS scores in men were lower (worse) than women (p =0.01), an effect that was more pronounced in men with greater dyspnea scores. In a similar analysis, SF-12 MCS scores in women were lower than men (worse) (48.3 vs. 54.4, p =0.0004), an effect that was more pronounced in women with greater dyspnea scores. Conclusions: Significant gender differences in HRQL exist in IPF. As compared to women, men reported less severe dyspnea, had worse SF-12 PCS scores, but better SF-12 MCS scores. Dyspnea appears to have a greater impact on the physical HRQL of men and the emotional HRQL of women. An improved understanding of the mechanism behind these differences is needed to better target interventions. [Copyright &y& Elsevier]