Human T-cell leukaemia virus type 1 associated pulmonary disease: clinical and pathological features of an under-recognised complication of HTLV-1 infection.

The lung is one of several organs that can be affected by HTLV-1 mediated inflammation. Pulmonary inflammation associated with HTLV-1 infection involves the interstitium, airways and alveoli, resulting in several clinical entities including interstitial pneumonias, bronchiolitis and alveolitis, depending on which structures are most affected. Augmentation of the inflammatory effects of HTLV-1 infected lymphocytes by recruitment of other inflammatory cells in a positive feedback loop is likely to underlie the pathogenesis of HTLV-1 associated pulmonary disease, as has been proposed for HTLV-1 associated myelopathy. In contrast to the conclusions of early case series, HTLV-1 associated pulmonary disease can be associated with significant parenchymal damage, which may progress to bronchiectasis where this involves the airways. Based on our current understanding of HTLV-1 associated pulmonary disease, diagnostic criteria are proposed.

Occupational lung diseases in the 21st century: the changing landscape and future challenges.

PURPOSE OF REVIEW: Occupational exposures remain an underrecognized and preventable cause of lung disease in high-income countries. The present review highlights the emergence of cleaning-related respiratory disease and the re-emergence of silicosis as examples of trends in occupational lung diseases in the 21st century. RECENT FINDINGS: Employment trends, such as the shift from large-scale manufacturing to a service economy, the growth of the healthcare sector, and changing consumer products have changed the spectrum of work-related lung diseases. Following decades of progress in reducing traditional hazards such as silica in U.S. workplaces, cases of advanced silicosis have recently re-emerged with the production of engineered stone countertops. With growth in the healthcare and service sectors in the United States, cleaning products have become an important cause of work-related asthma and have recently been associated with an increased risk of chronic obstructive pulmonary disease (COPD) in women. However, these occupational lung diseases largely go unrecognized by practicing clinicians. SUMMARY: The present article highlights how changes in the economy and work structure can lead to new patterns of inhalational workplace hazards and respiratory disease, including cleaning-related respiratory disease and silicosis. Pulmonary clinicians need to be able to recognize and diagnose these occupational lung diseases, which requires a high index of suspicion and a careful occupational history.

Classification of pulmonary lesion based on multiparametric MRI: utility of radiomics and comparison of machine learning methods.

OBJECTIVES: We develop and validate a radiomics model based on multiparametric magnetic resonance imaging (MRI) in the classification of the pulmonary lesion and identify optimal machine learning methods. MATERIALS AND METHODS: This retrospective analysis included 201 patients (143 malignancies, 58 benign lesions). Radiomics features were extracted from multiparametric MRI, including T2-weighted imaging (T2WI), T1-weighted imaging (TIWI), and apparent diffusion coefficient (ADC) map. Three feature selection methods, including recursive feature elimination (RFE), t test, and least absolute shrinkage and selection operator (LASSO), and three classification methods, including linear discriminate analysis (LDA), support vector machine (SVM), and random forest (RF) were used to distinguish benign and malignant pulmonary lesions. Performance was compared by AUC, sensitivity, accuracy, precision, and specificity. Analysis of performance differences in three randomly drawn cross-validation sets verified the stability of the results. RESULTS: For most single MR sequences or combinations of multiple MR sequences, RFE feature selection method with SVM classifier had the best performance, followed by RFE with RF. The radiomics model based on multiple sequences showed a higher diagnostic accuracy than single sequence for every machine learning method. Using RFE with SVM, the joint model of T1WI, T2WI, and ADC showed the highest performance with AUC = 0.88 ± 0.02 (sensitivity 83%; accuracy 82%; precision 91%; specificity 79%) in test set. CONCLUSION: Quantitative radiomics features based on multiparametric MRI have good performance in differentiating lung malignancies and benign lesions. The machine learning method of RFE with SVM is superior to the combination of other feature selection and classifier methods. KEY POINTS: • Radiomics approach has the potential to distinguish between benign and malignant pulmonary lesions. • Radiomics model based on multiparametric MRI has better performance than single-sequence models. • The machine learning methods RFE with SVM perform best in the current cohort.

A Lung Ultrasound Severity Score Predicts Chronic Lung Disease in Preterm Infants.

OBJECTIVE: To test the hypothesis that a lung ultrasound severity score (LUSsc) can predict the development of chronic lung disease (CLD) in preterm neonates. STUDY DESIGN: Preterm infants <30 weeks' gestational age were enrolled in this study. Lung ultrasound (LUS) was performed between 1 and 9 postnatal weeks. All ultrasound studies were done assessing three lung zones on each lung. Each zone was given a score between 0 and 3. A receiver operating characteristic curve was constructed to assess the ability of LUSsc to predict CLD. RESULTS: We studied 27 infants at a median (interquartile range [IQR]) gestational age and birth weight of 26 weeks (25-29) and 780 g (530-1,045), respectively. Median (IQR) postnatal age at the time of LUS studies was 5 (2-8) weeks. Fourteen infants who developed CLD underwent 34 studies. Thirteen infants without CLD underwent 30 studies. Those who developed CLD had a higher LUSsc than those who did not (median [IQR] of scores: 9 [6-12] vs. 3 [1-4], p < 0.0001). An LUSsc cutoff of 6 has a sensitivity and specificity of 76 and 97% and positive and negative predictive values of 95 and 82%, respectively. Adding gestational age < 27 weeks improved sensitivity and specificity to 86 and 98% and positive and negative predictive values to 97 and 88%. CONCLUSION: LUSsc between 2 and 8 weeks can predict development of CLD in preterm neonates.

The Effect of Pulmonary Rehabilitation on Non-chronic Obstructive Pulmonary Disease Patients.

BACKGROUND: Pulmonary rehabilitation has shown significant benefit for patients with chronic obstructive pulmonary disease (COPD). The effect on non-COPD pulmonary patients is less well established. OBJECTIVES: To determine whether pulmonary rehabilitation is also beneficial for non-COPD pulmonary patients. METHODS: Clinical and demographic data on non-COPD pulmonary patients who participated in our institutional pulmonary rehabilitation program between January 2009 and December 2016 were collected. Participants engaged in a 60-minute, twice-weekly, ambulatory hospital-based program lasting 12 to 24 sessions. Sessions included both endurance and muscle training as well as healthy lifestyle educational activities. The six-minute walk test (6MWT) and the St. George's Respiratory Questionnaire (SGRQ) were conducted before and after the rehabilitation program. RESULTS: We recruited 214 non-COPD patients, of whom 153 completed at least 12 sessions. Of these, 59 presented with interstitial lung disease (ILD), 18 with non-ILD restrictive lung defects, 25 with asthma, 30 with lung cancer, and 21 with other conditions (e.g., pulmonary hypertension, bronchiectasis) The groups demonstrated significant improvement in 6MWT and in SGRQ scores. Non-COPD patients gained a 61.9 meter (19%) improvement in the 6MWT (P < 0.0001) and 8.3 point reduction in their SGRQ score (P < 0.0001). CONCLUSIONS: Pulmonary rehabilitation is effective in non-COPD pulmonary patients. As such, it should be an integral part of the treatment armament provided to the vast majority of those suffering from chronic respiratory disease.

CT features of lung agenesis - a case series (6 cases).

BACK GROUND: Lung agenesis is a rare congenital anomaly. The main etiology of the disease is unknown whereas genetic, iatrogenic and viral factors as well as vitamin A deficiency during early pregnancy may result in developmental failure of primitive lung bud causing unilateral pulmonary agenesis. Affected patients usually present with variable respiratory symptoms and recurrent chest infection at any age. Plain film demonstrates opaque unilateral lung while chest CT scan can definitely diagnosis the disease. The anomaly has three types. Type I is pulmonary agenesis, type II is called pulmonary aplasia and type III is pulmonary hypoplasia. CASES' PRESENTATION: Six patients with main complaint of dyspnea underwent contrast enhanced chest CT in radiology department of French Medical Institute for Mothers and children, Kabul and were diagnosed lung agenesis. Three patients were categorized as type II pulmonary agenesis (aplasia). Two patients, three months old boy and a seven year- old girl demonstrated right lung aplasia. Another patient boy of eighteen years old presented with left lung aplasia. Two boys of four and seven months of age were classified as type I pulmonary agenesis (agenesis). A boy of one year old was diagnosed pulmonary agenesis type III, right lung hypoplasia. CONCLUSION: Six patients were diagnosed with pulmonary agenesis by Chest CT scan. The clinicians should consider possibility of congenital pulmonary agenesis in dyspneic patients with opaque unilateral hemithorax in plain film.

Pulmonary infections in immunocompromised patients: the role of image-guided fine needle aspiration cytology.

OBJECTIVE: To evaluate the role of fine needle aspiration cytology (FNAC) in the diagnosis of pulmonary infections in immunocompromised patients and to identify the imaging pattern of infections on computed tomography (CT). MATERIALS AND METHODS: This was a retrospective study of 42 immunocompromised patients who underwent FNAC under image guidance owing to a clinical pulmonary infection. Each patient was evaluated for an underlying immunocompromised condition, cytological diagnosis, CT findings and complications. RESULTS: The most common predisposing condition was diabetes mellitus (n = 11), renal transplant status (n = 11) followed by connective tissue disorders (n = 6) and malignancy (n = 5). There were four patients with renal disease and three had a human immunodeficiency virus (HIV) infection. The most common cytological diagnosis was mucormycosis (n = 13) followed by nocardiosis (n = 8) and necrotising inflammation (n = 7), tuberculosis (n = 6), cryptococcosis (n = 2), aspergillosis(n = 2), histoplasmosis(n = 1) and atypical mycobacterial infection (n = 1). Mucormycosis presented as a pulmonary nodule (n = 7), mass lesion (n = 5) or consolidation (n = 4). The patients with nocardiosis had lung nodules with associated consolidation and cavitation. None of the patients had any major complication. CONCLUSION: FNA is a relatively reliable, safe and quick method of diagnosing pulmonary infection in immunocompromised patients. Cytomorphological features, when aided by special stains, can accurately detect the specific infection which is potentially treatable. Specific infections may be suggested based on specific imaging patterns.

A review of congenital lung malformations with a simplified classification system for clinical and research use.

PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. RESULTS: The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. CONCLUSIONS: Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.

Potential Clinical Utility of FDG-PET in Non-malignant Pulmonary Disorders: A Pilot Study.

BACKGROUND: Fluorodeoxyglucose (FDG) positron emission tomography (PET) is emerging as an important non- invasive investigation in benign pulmonary conditions too. The aim of this study was to investigate its utility in the diagnosis and monitoring of various benign pulmonary diseases. METHODS: In this prospective observational hospital-based study 50 consecutive patients (26 males) with benign lung diseases underwent computed tomography of chest followed by FDG-PET at baseline and after treatment where appropriate. The findings of FDG scan are reported in the context of clinical, histopathological, physiological and radiological findings. RESULTS: All patients showed increased FDG uptake in the lung corresponding to CT findings. Of the 9 patients with sarcoidosis stage 1 (n=1), stage 2 (n=3) and stage 3 (n=5), additional uptake in the myocardium and thyroid was noted in two patients which resulted in a change in the modality of treatment. Repeat FDG scan post-treatment showed decreased uptake in all patients which was consistent with clinico-radiologic, microbiological or spirometry findings. Increased uptake was seen in one patient with pulmonary tuberculosis (TB) and in one patient with TB mediastinal lymphadenopathy at the end of intensive phase discordant with clinical and microbiological response. Of nine cases of idiopathic interstitial pneumonias (IIPs), additional intense FDG uptake was found in two cases which corresponded to the areas of honeycombing. CONCLUSIONS: FDG-PET scan can be used as an important adjunct non-invasive investigation in diagnosing and monitoring of various benign lung conditions. It also helps in assessing whole body disease burden which may change therapeutic decisions.

Severe pulmonary hypertension in lung disease: phenotypes and response to treatment.

Pulmonary hypertension (PH) due to lung disease (World Health Organization (WHO) group 3) is common, but severe PH, arbitrarily defined as mean pulmonary artery pressure ≥35 mmHg is reported in only a small proportion. Whether these should be treated as patients in WHO group 1 (i.e. pulmonary arterial hypertension) with PH-targeted therapies is unknown. We compared the phenotypic characteristics and outcomes of 118 incident patients with severe PH and lung disease with 74 idiopathic pulmonary arterial hypertension (IPAH) patients, all treated with pulmonary vasodilators. Lung disease patients were older, more hypoxaemic, and had lower gas transfer, worse New York Heart Association functional class and lower 6-min walking distance (6MWD) than IPAH patients. Poorer survival in those with lung disease was driven by the interstitial lung disease (ILD) cohort. In contrast to IPAH, where significant improvements in 6MWD and N-terminal pro-brain natruiretic peptide (NT-proBNP) occurred, PH therapy in severe PH lung disease did not lead to improvement in 6MWD or functional class, but neither was deterioration seen. NT-proBNP decreased from 2200 to 1596 pg·mL(-1) (p=0.015). Response varied by lung disease phenotype, with poorer outcomes in patients with ILD and emphysema with preserved forced expiratory volume in 1 s. Further study is required to investigate whether vasodilator therapy may delay disease progression in severe PH with lung disease.

[Pulmonary manifestations in rheumatoid arthritis]. / Manifestacje plucne w reumatoidalnym zapaleniu stawów.

Rheumatoid arthritis (RA) is a chronic inflammatory disease characterized by destructive cartilages, bones and other structures formed joints. RA belongs to connective tissue diseases represented by systemic nature, internal illness, extra-articular features and rapidly progress of atherosceirosis. The extra-articular complications cause the reduction of patient longevity. The frequency of symptoms in patient with RA and respiratory disorders occur in 10-20% of cases. Pulmonary complications are the second most common cause of premature of patient deaths. Respiratory disorders associated with RA are devided into 3 groups: infection, lung disease caused by drugs and pulmonary manifestation connected by RA. These last affect interstitial tissue, bronchioli, pulmonary vessels, pleura, also are presented by pulmonary rheumatoid nodules and pulmonary hypertension.

Congenital pulmonary lymphangiectasis.

Congenital pulmonary lymphangiectasis (CPL) is a rare vascular malformation causing dilated lymph vessels and disturbed drainage of lymph fluid. Based on the pathogenesis and clinical phenotype it can be classified as primary or secondary CPL. Associated genetic syndromes with or without lymphedema, familial occurrence and gene mutations have been described. In utero, it may present as non-immune hydrops with pleural effusions. At birth neonates may have respiratory failure due to chylothorax and pulmonary hypoplasia, causing very high short term mortality rates. Other cases may become symptomatic any time later in childhood or even during adult life. CPL is usually diagnosed based on the combination of clinical signs, imaging and histological findings. Open-lung biopsy is considered the gold standard for the diagnosis of CPL. Treatment is primarily supportive featuring aggressive mechanical ventilation and the management of problems associated with congenital chylothorax including chest-drainage, medium-chain triglycerides (MCT) diet, and octreotide.

Lung transplantation in adults and children: putting lung function into perspective.

The number of lung transplants performed globally continues to increase year after year. Despite this growing experience, long-term outcomes following lung transplantation continue to fall far short of that described in other solid-organ transplant settings. Chronic lung allograft dysfunction (CLAD) remains common and is the end result of exposure to a multitude of potentially injurious insults that include alloreactivity and infection among others. Central to any description of the clinical performance of the transplanted lung is an assessment of its physiology by pulmonary function testing. Spirometry and the evaluation of forced expiratory volume in 1 s and forced vital capacity, remain core indices that are measured as part of routine clinical follow-up. Spirometry, while reproducible in detecting lung allograft dysfunction, lacks specificity in differentiating the different complications of lung transplantation such as rejection, infection and bronchiolitis obliterans. However, interpretation of spirometry is central to defining the different 'chronic rejection' phenotypes. It is becoming apparent that the maximal lung function achieved following transplantation, as measured by spirometry, is influenced by a number of donor and recipient factors as well as the type of surgery performed (single vs double vs lobar lung transplant). In this review, we discuss the wide range of variables that need to be considered when interpreting lung function testing in lung transplant recipients. Finally, we review a number of novel measurements of pulmonary function that may in the future serve as better biomarkers to detect and diagnose the cause of the failing lung allograft.

[Cardiopulmonary stress test--interpretation and clinical indications].

A recent report in Harefuah has introduced cardiopulmonary exercise testing (CPX), presenting its physiological basis and key parameters. Despite the fact that multiple guideline documents and scientific statements have been published in the last few years by the leading European and American societies summarizing the incremental information added by the addition of ventilatory gas exchange measurements, CPX continues to be underutilized by the practicing clinician. One of the main reasons for this is the lack of understanding of the value of CPX by the practicing clinicians. In this review we will describe the current clinical and emerging applications of CPX, and try to simplify interpretation and reporting of CPX test data. We will also review CPX findings in selected clinical populations and the implication of these observations to the clinical evaluation of patients with heart and/ or lung diseases.

Chest CT Image based Lung Disease Classification - A Review.

Computed tomography (CT) scans are widely used to diagnose lung conditions due to their ability to provide a detailed overview of the body's respiratory system. Despite its popularity, visual examination of CT scan images can lead to misinterpretations that impede a timely diagnosis. Utilizing technology to evaluate images for disease detection is also a challenge. As a result, there is a significant demand for more advanced systems that can accurately classify lung diseases from CT scan images. In this work, we provide an extensive analysis of different approaches and their performances that can help young researchers to build more advanced systems. First, we briefly introduce diagnosis and treatment procedures for various lung diseases. Then, a brief description of existing methods used for the classification of lung diseases is presented. Later, an overview of the general procedures for lung disease classification using machine learning (ML) is provided. Furthermore, an overview of recent progress in ML-based classification of lung diseases is provided. Finally, existing challenges in ML techniques are presented. It is concluded that deep learning techniques have revolutionized the early identification of lung disorders. We expect that this work will equip medical professionals with the awareness they require in order to recognize and classify certain medical disorders.

Segmenting and classifying lung diseases with M-Segnet and Hybrid Squeezenet-CNN architecture on CT images.

Diagnosing lung diseases accurately and promptly is essential for effectively managing this significant public health challenge on a global scale. This paper introduces a new framework called Modified Segnet-based Lung Disease Segmentation and Severity Classification (MSLDSSC). The MSLDSSC model comprises four phases: "preprocessing, segmentation, feature extraction, and classification." Initially, the input image undergoes preprocessing using an improved Wiener filter technique. This technique estimates the power spectral density of the noisy and original images and computes the SNR assisted by PSNR to evaluate image quality. Next, the preprocessed image undergoes Segmentation to identify and separate the RoI from the background objects in the lung image. We employ a Modified Segnet mechanism that utilizes a proposed hard tanh-Softplus activation function for effective Segmentation. Following Segmentation, features such as MLDN, entropy with MRELBP, shape features, and deep features are extracted. Following the feature extraction phase, the retrieved feature set is input into a hybrid severity classification model. This hybrid model comprises two classifiers: SDPA-Squeezenet and DCNN. These classifiers train on the retrieved feature set and effectively classify the severity level of lung diseases.

Classification of diffuse lung diseases: why and how.

The understanding of complex lung diseases, notably the idiopathic interstitial pneumonias and small airways diseases, owes as much to repeated attempts over the years to classify them as to any single conceptual breakthrough. One of the many benefits of a successful classification scheme is that it allows workers, within and between disciplines, to be clear that they are discussing the same disease. This may be of particular importance in the recruitment of individuals for a clinical trial that requires a standardized and homogeneous study population. Different specialties require fundamentally different things from a classification: for epidemiologic studies, a classification that requires categorization of individuals according to histopathologic pattern is not usually practicable. Conversely, a scheme that simply divides diffuse parenchymal disease into inflammatory and noninflammatory categories is unlikely to further the understanding about the pathogenesis of disease. Thus, for some disease groupings, for example, pulmonary vasculopathies, there may be several appropriate classifications, each with its merits and demerits. There has been an interesting shift in the past few years, from the accepted primacy of histopathology as the sole basis on which the classification of parenchymal lung disease has rested, to new ways of considering how these entities relate to each other. Some inventive thinking has resulted in new classifications that undoubtedly benefit patients and clinicians in their endeavor to improve management and outcome. The challenge of understanding the logic behind current classifications and their shortcomings are explored in various examples of lung diseases.

Limited pulmonary MPA, a new MPA entity? A rheumatologist's perspective.

Microscopic polyangiitis (MPA) frequently involves the lungs. However, as opposed to granulomatosis with polyangiitis (Wegener's), limited forms are not recognised. In recent years, cases have been reported in which the lungs were affected without other organ manifestations. For years, many have been labelled as idiopathic pulmonary fibrosis (IPF). In this review, support for the existence of a limited form of MPA affecting the lungs as well as questions and discussions concerning the similarities and differences to IPF are offered.

[Scores and stages in pneumology]. / Scores und Stadien in der Pneumologie.

Useful scales and classifications for patients with pulmonary diseases are discussed. The modified Medical Research Council breathlessness scale (mMRC) is a measure of disability in lung patients. The GOLD classifications, the COPD-Assessment Test (CAT) and the BODE Index are important to classify the severity of COPD and to measure the disability of these patients. The Geneva score is a clinical prediction rule used in determining the pre-test probability of pulmonary embolism. The Pulmonary Embolism Severity Index (PESI) is a scoring system used to predict 30 day mortality in patients with pulmonary embolism. The Epworth Sleepiness Scale is intended to measure daytime sleepiness in patients with sleep apnea syndrome. The Asthma Controll Test (ACT) determines if asthma symptoms are well controlled.

Lymphocytic interstitial pneumonia and other benign lymphoid disorders.

Nonneoplastic pulmonary lymphoid disorders consist of a complex spectrum of diseases for pathologists and pulmonologists alike. Advances in our understanding of these disorders in recent years have led to revisions in the classification scheme. This review summarizes the clinicoradiological and pathological features of several benign pulmonary lymphoid disorders as well as the current knowledge regarding their pathogenesis. The disorders discussed include lymphocytic interstitial pneumonitis, follicular bronchiolitis, nodular lymphoid hyperplasia, inflammatory pseudotumor, Castleman disease, immunoglobulin G4-related disease in the lung, and posttransplant lymphoproliferative disease.