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Rationale: Pulmonary artery sarcoma (PAS) is a rare malignant tumor primarily originating from the pulmonary artery's intima or subintima. Approximately one-third of cases are classified as undifferentiated type. Its clinical manifestations lack specificity, dyspnea is the main symptom but can also present with chest pain, cough, hemoptysis, and other discomforts, making it prone to misdiagnosis as pulmonary embolism (PE)., Patient Concerns: A 50-year-old woman was admitted to the hospital with "dyspnea for more than 3 months, aggravated for 2 days," and computed tomography pulmonary angiography suggesting "bilateral multiple pulmonary embolisms.", Diagnoses: The patient was initially misdiagnosed as PE, and was later definitively diagnosed as undifferentiated pleomorphic sarcoma of the pulmonary artery by pathologic biopsy., Interventions and Outcomes: The patient was initially treated with anticoagulant therapy, but her dyspnea was not relieved. After that, she underwent positron emission computed tomography (PET-CT) and other investigations, which suggested the possibility of PAS, and then she underwent pulmonary endarterectomy to remove the lesion, which relieved her symptoms and was advised to seek further medical attention from the Department of Oncology and Department of Radiotherapy., Lessons: PAS can be easily misdiagnosed as PE. If a diagnosis of PE is made, but anticoagulation or even thrombolytic therapy proves ineffective, and there is no presence of PE causative factors such as deep vein thrombosis in the lower extremities, or D-dimer levels are not high, one should be cautious and consider the possibility of PAS., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

A 7-year-old Icelandic gelding was presented with acute severe dyspnea of one day duration and purulent nasal discharge that had been present for 6 weeks. Clinically, the initial examination focused on severe enlargement of the mandibular and retropharyngeal lymph nodes as well as a mixed dyspnea.The diagnosis of a malignant lymphoma was evident following laboratory diagnostics, endoscopy, and cytological examination of a fine needle aspiration of a mandibular lymph node. The gelding was euthanized due to the poor prognosis and a significantly disturbed general condition. Pathohistological examination revealed a multicentric T-cell-rich B-cell lymphoma., Competing Interests: Die Autoren bestätigen, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

Idiopathic obliterative bronchiolitis (OB) is a rare disease that usually requires a surgical lung biopsy. A 25-year-old woman with progressive exertional dyspnea for several months showed a severe mixed restrictive and obstructive pattern on spirometry. Chest computed tomography showed a mosaic pattern, and pulmonary ventilation-perfusion scintigraphy showed a matched defect. The bronchoscopic specimens obtained from both the alveolar and bronchiolar regions of the predicted lesion area contributed to the diagnosis of OB. She had no underlying causes of secondary OB, and she was diagnosed with idiopathic OB. Since lung transplantation was indicated, she was referred to a lung transplantation-certified hospital.

Objective: To analyze the clinical characteristics and prognosis of 6 children with idiopathic interstitial pneumonia (IIP). Methods: This retrospective study analyzed the clinical manifestations, examinations, treatment and prognosis of 6 children with IIP who were hospitalized in Children's Hospital of Nanjing Medical University from January 2015 to March 2020. Results: Of the 6 children, 2 were males and 4 were females, aged 4.8 to10.6 years. All children had a subacute onset, and presented with cough, shortness of breath and cyanosis. The lung high-resolution CT (HRCT) showed diffuse patchiness in bilateral lung fields in all the children and reticular pattern in 2 cases. Pulmonary function test found moderate to severe mixed defect in 5 children. Lung biopsy was performed in 4 children. All of the 6 children were treated with systemic glucocorticoids, of whom 2 cases had additional inhaled glucocorticoids. Four children were finally diagnosed as cryptogenic organizing pneumonia (COP), whose lung HRCT return to normal in 1-11 months. Two children were finally diagnosed as nonspecific interstitial pneumonia (NSIP), and had long-term residual fibrosis on lung HRCT. The 6 children were followed up for 1 year to 6 years and 5 months after discontinuation of systemic glucocorticoids, and all had no recurrence. Conclusions: The clinical characteristics of IIP in children are subacute onset presented with cough, shortness of breath, cyanosis and diffuse patchiness in bilateral lungs on HRCT. The common subtypes of IIP in children are COP and NSIP. Systemic glucocorticoid is effective for IIP in children and there is a good prognosis overall.

Primary adenoid cystic carcinoma (ACC) of the trachea is a rare entity, with a 5-year survival between 50% and 80% for resectable cases and 30% in case of unresectable disease. We report a case of a primary ACC on a woman in her 70s that presented with a drawn-out history of dyspnoea. She was diagnosed with an unresectable obstructive tumour of the trachea, which required the placement of a Y-shaped stent. The patient underwent concomitant chemoradiotherapy, with partial response, and is still in follow-up, without evidence of disease progression., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Importance: When hospitals are at capacity, accurate deterioration indices could help identify low-risk patients as potential candidates for home care programs and alleviate hospital strain. To date, many existing deterioration indices are based entirely on structured data from the electronic health record (EHR) and ignore potentially useful information from other sources., Objective: To improve the accuracy of existing deterioration indices by incorporating unstructured imaging data from chest radiographs., Design, Setting, and Participants: Machine learning models were trained to predict deterioration of patients hospitalized with acute dyspnea using existing deterioration index scores and chest radiographs. Models were trained on hospitalized patients without coronavirus disease 2019 (COVID-19) and then subsequently tested on patients with COVID-19 between January 2020 and December 2020 at a single tertiary care center who had at least one radiograph taken within 48 hours of hospital admission., Main Outcomes and Measures: Patient deterioration was defined as the need for invasive or non-invasive mechanical ventilation, heated high flow nasal cannula, IV vasopressor administration or in-hospital mortality at any time following admission. The EPIC deterioration index was augmented with unstructured data from chest radiographs to predict risk of deterioration. We compared discriminative performance of the models with and without incorporating chest radiographs using area under the receiver operating curve (AUROC), focusing on comparing the fraction and total patients identified as low risk at different negative predictive values (NPV)., Results: Data from 6278 hospitalizations were analyzed, including 5562 hospitalizations without COVID-19 (training cohort) and 716 with COVID-19 (216 in validation, 500 in held-out test cohort). At a NPV of 0.95, the best-performing image-augmented deterioration index identified 49 more (9.8%) individuals as low-risk compared to the deterioration index based on clinical data alone in the first 48 hours of admission. At a NPV of 0.9, the EPIC image-augmented deterioration index identified 26 more individuals (5.2%) as low-risk compared to the deterioration index based on clinical data alone in the first 48 hours of admission., Conclusion and Relevance: Augmenting existing deterioration indices with chest radiographs results in better identification of low-risk patients. The model augmentation strategy could be used in the future to incorporate other forms of unstructured data into existing disease models., Competing Interests: The authors have declared that no competing interests exist.

The ongoing Covid-19 is a contagious disease, and it is characterised by different symptoms such as fever, cough, and shortness of breath. Rising concerns about Covid-19 have severely affected the healthcare system in all countries as the Covid-19 outbreak has developed at a rapid rate all around the globe. Intriguing, a clinically used drug, acetazolamide (a specific inhibitor of carbonic anhydrase, CA, EC 4.2.1.1), is used to treat high-altitude pulmonary oedema (HAPE), showing a high degree of clinical similarities with the pulmonary disease caused by Covid-19. In this context, this preliminary study aims to provide insights into some factors affecting the Covid-19 patients, such as hypoxaemia, hypoxia as well as the blood CA activity. We hypothesise that patients with Covid-19 problems could show a dysregulated acid-base status influenced by CA activity. These preliminary results suggest that the use of CA inhibitors as a pharmacological treatment for Covid-19 may be beneficial.

SARS Coronavirus-2 is one of the most widespread viruses globally during the 21 st century, whose severity and ability to cause severe pneumonia and death vary. We performed a comprehensive systematic review of all studies that met our standardised criteria and then extracted data on the age, symptoms, and different treatments of Covid-19 patients and the prognosis of this disease during follow-up. Cases in this study were divided according to severity and death status and meta-analysed separately using raw mean and single proportion methods. We included 171 complete studies including 62,909 confirmed cases of Covid-19, of which 148 studies were meta-analysed. Symptoms clearly emerged in an escalating manner from mild-moderate symptoms, pneumonia, severe-critical to the group of non-survivors. Hypertension (Pooled proportion (PP): 0.48 [95% Confident interval (CI): 0.35-0.61]), diabetes (PP: 0.23 [95% CI: 0.16-0.33]) and smoking (PP: 0.12 [95% CI: 0.03-0.38]) were highest regarding pre-infection comorbidities in the non-survivor group. While acute respiratory distress syndrome (PP: 0.49 [95% CI: 0.29-0.78]), (PP: 0.63 [95% CI: 0.34-0.97]) remained one of the most common complications in the severe and death group respectively. Bilateral ground-glass opacification (PP: 0.68 [95% CI: 0.59-0.75]) was the most visible radiological image. The mortality rates estimated (PP: 0.11 [95% CI: 0.06-0.19]), (PP: 0.03 [95% CI: 0.01-0.05]), and (PP: 0.01 [95% CI: 0-0.3]) in severe-critical, pneumonia and mild-moderate groups respectively. This study can serve as a high evidence guideline for different clinical presentations of Covid-19, graded from mild to severe, and for special forms like pneumonia and death groups., (© 2021 John Wiley & Sons Ltd.)

Competing Interests: The authors declare no conflict of interest.

Background: The prevalence of pulmonary embolism (PE) in the acute exacerbation of chronic obstructive pulmonary disease (AE-COPD) is highly controversial. We conducted a systematic review and meta-analysis to summarize the epidemiology and characteristics of PE with AE-COPD for current studies., Methods: We searched the PubMed, EMBASE, Cochrane Library and Web of Science databases for studies published prior to October 21, 2020. Pooled proportions with 95% confidence intervals (95% CIs) were calculated using a random effects model. Odds ratios (ORs) and mean differences (MDs) with 95% confidence intervals were used as effect measures for dichotomous and continuous variables, respectively., Results: A total of 17 studies involving 3170 patients were included. The prevalence of PE and deep vein thrombosis (DVT) in AE-COPD patients was 17.2% (95% CI: 13.4%-21.3%) and 7.1% (95% CI: 3.7%-11.4%%), respectively. Dyspnea (OR = 6.77, 95% CI: 1.97-23.22), pleuritic chest pain (OR = 3.25, 95% CI: 2.06-5.12), lower limb asymmetry or edema (OR = 2.46, 95% CI:1.51-4.00), higher heart rates (MD = 20.51, 95% CI: 4.95-36.08), longer hospital stays (MD = 3.66, 95% CI: 3.01-4.31) were associated with the PE in the AE-COPD patients. Levels of D-dimer (MD = 1.51, 95% CI: 0.80-2.23), WBC counts (MD = 1.42, 95% CI: 0.14-2.70) were significantly higher and levels of PaO2 was lower (MD = -17.20, 95% CI: -33.94- -0.45, P<0.05) in the AE-COPD with PE group. The AE-COPD with PE group had increased risk of fatal outcome than the AE-COPD group (OR = 2.23, 95% CI: 1.43-3.50)., Conclusions: The prevalence of PE during AE-COPD varies considerably among the studies. AE-COPD patients with PE experienced an increased risk of death, especially among the ICU patients. Understanding the potential risk factors for PE may help clinicians identify AE-COPD patients at increased risk of PE., Prospero Registration Number: CRD42021226568., Competing Interests: The authors have declared that no competing interests exist.

The natural history of COVID-19 and predictors of mortality in older adults need to be investigated to inform clinical operations and healthcare policy planning. A retrospective study took place in 80 long-term nursing homes in Catalonia, Spain collecting data from March 1st to May 31st, 2020. Demographic and clinical data from 2,092 RT-PCR confirmed cases of SARS-CoV-2 infection were registered, including structural characteristics of the facilities. Descriptive statistics to describe the demographic, clinical, and molecular characteristics of our sample were prepared, both overall and by their symptomatology was performed and an analysis of statistically significant bivariate differences and constructions of a logistic regression model were carried out to assess the relationship between variables. The incidence of the infection was 28%. 71% of the residents showed symptoms. Five major symptoms included: fever, dyspnea, dry cough, asthenia and diarrhea. Fever and dyspnea were by far the most frequent (50% and 28%, respectively). The presentation was predominantly acute and symptomatology persisted from days to weeks (mean 9.1 days, SD = 10,9). 16% of residents had confirmed pneumonia and 22% required hospitalization. The accumulated mortality rate was 21.75% (86% concentrated during the first 28 days at onset). A multivariate logistic regression analysis showed a positive predictive value for mortality for some variables such as age, pneumonia, fever, dyspnea, stupor refusal to oral intake and dementia (p<0.01 for all variables). Results suggest that density in the nursing homes did not account for differences in the incidence of the infection within the facilities. This study provides insights into the natural history of the disease in older adults with high dependency living in long-term nursing homes during the first pandemic wave of March-May 2020 in the region of Catalonia, and suggests that some comorbidities and symptoms have a strong predictive value for mortality., Competing Interests: The authors have declared that no competing interests exist.

Polymerase chain reaction (PCR)-based diagnostics for Mycoplasma pneumoniae (M. pneumoniae) from the respiratory tract has become widely available, but the interpretation of the results remains unclear. M. pneumoniae has been suggested to cause mainly mild and self-limiting infections or asymptomatic carriage. However, systematic analyses of the association between PCR results and clinical findings are scarce. This study aimed to clarify the clinical features of PCR-positive M. pneumoniae infections in a hospital setting. We reviewed 103 PCR-positive patients cared for in a university hospital during a 3-year period. Data on age, sex, health condition, acute symptoms, other pathogens found, laboratory and X-ray results and treatments were collected. Over 85% of the patients had a triad of typical symptoms: fever, cough and shortness of breath. Symptoms in the upper respiratory tract were rare. In 91% of the cases, M. pneumoniae was the only pathogen found. The highest incidence was found in the age group of 30-40 years, and 68% of the patients did not have any underlying diseases. Most patients were initially empirically treated with beta-lactam antibiotics and needed 2-4 changes in their treatment. Only 6% were discharged without an antibiotic effective against M. pneumoniae. This study shows that M. pneumoniae often led to hospitalisation and that patients needed appropriate antimicrobial treatment to recover. Mixed infections were rare, and situations that could be interpreted as carriage did not occur.

Introduction: Patients at intermediate-high risk of developing a pulmonary embolism (PE) are very likely to experience adverse outcomes, such as cardiovascular instability and death. The role of thrombolytic therapy in intermediate-high-risk PE remains controversial., Objectives: This study aimed to determine the efficacy and safety of low-dose urokinase (UK) thrombolytic therapy for intermediate-high-risk PE., Patients and Methods: This retrospective study included 81 consecutive patients with intermediate-high-risk PE from two centers. Patients received low-dose UK or low-molecular-weight heparin (anticoagulant therapy group). The efficacy outcomes were mortality, computed tomography pulmonary angiography (CTPA)-confirmed absorption, and dyspnea. Safety was assessed as the incidence of bleedings., Results: The in-hospital mortality, 9-month mortality, and long-term mortality at the last follow-up were comparable for the low-dose UK group and the anticoagulant therapy group (6.45% vs. 0%, p = 0.144, 9.68% vs. 8.16%, p = 0.815, and 12.90% vs. 12.24%, p = 0.931, respectively). CTPA-confirmed absorption at one month after admission was higher in the low-dose UK group than in the anticoagulant therapy group (p = 0.016). The incidences of short-term dyspnea at discharge and long-term dyspnea at the last follow-up were lower in the low-dose UK group than in the anticoagulant therapy group (27.59% vs. 52%, p = 0.035, 33.33% vs. 58.14%, p = 0.043, respectively). No major bleeding occurred. The incidence of minor bleeding was not significantly different between the two groups (3.23% vs. 6%, p = 0.974)., Conclusion: In intermediate-high-risk PE, a low-dose UK might increase CTPA-confirmed absorption and improve short-term and long-term dyspnea without affecting mortality or increasing the bleeding risk., Competing Interests: The authors declare no conflict of interest.

Dyspnoea is a cardinal symptom of fibrotic interstitial lung disease (ILD), with a lack of proven effective therapies. With emerging evidence of the role of facial and nasal airflow for relieving breathlessness, this pilot study was conducted to examine the feasibility of conducting a clinical trial of a handheld fan (HHF) for dyspnoea management in patients with fibrotic ILD. In this mixed-methods, randomised, assessor-blinded, controlled trial, 30 participants with fibrotic ILD who were dyspnoeic with a modified Medical Research Council Dyspnoea grade ≥ 2 were randomised to a HHF for symptom control or no intervention for 2 weeks. Primary outcomes were trial feasibility, change in Dyspnoea-12 scores at Week 2, and participants' perspectives on using a HHF for dyspnoea management. Study recruitment was completed within nine months at a single site. Successful assessor blinding was achieved in the fan group [Bang's Blinding Index - 0.08 (95% CI - 0.45, 0.30)] but not the control group [0.47 (0.12, 0.81)]. There were no significant between-group differences for the change in Dyspnoea-12 or secondary efficacy outcomes. During qualitative interviews, participants reported that using the HHF relieved breathlessness and provided relaxation, despite initial scepticism about its therapeutic benefit. Oxygen-experienced participants described the HHF being easier to use, but not as effective for symptomatic relief, compared to oxygen therapy. Our results confirmed the feasibility of a clinical trial of a HHF in fibrotic ILD. There was a high level of patient acceptance of a HHF for managing dyspnoea, with patients reporting both symptomatic benefits and ease of use.

To determine the distribution of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) respiratory viral loads (VL) during the acute phase of infection and their correlation with clinical presentation and inflammation-related biomarkers. Nasopharyngeal swabs from 453 adult SARS-CoV-2-infected patients from the Department of Infectious Diseases, Besançon, France, were collected at the time of admission or consultation for reverse transcriptase polymerase chain reaction (RT-PCR) analysis. Clinical information and concentrations of biological parameters (C-reactive protein [CRP], fibrinogen, lactate dehydrogenase [LDH], prealbumin) were noticed. Mean respiratory VL homogeneously decreased from 7.2 log 10 copies/ml (95% confidence interval [CI]: 6.6-7.8) on the first day of symptoms until 4.6 log 10 copies/ml (95% CI: 3.8-5.4) at day 10 (slope = -0.24; R 2  = .95). VL were poorly correlated with COVID-19 symptoms and outcome, excepted for dyspnea and anosmia, which were significantly associated with lower VL (p < .05). CRP, fibrinogen, and LDH concentrations significantly increased over the first 10 days (median CRP concentrations from 36.8 mg/L at days 0-1 to 99.5 mg/L at days 8-10; p < .01), whereas prealbumin concentrations tended to decrease. Since SARS-CoV-2 respiratory VL regularly decrease in the acute phase of infection, determining the level of VL may help predicting the onset of virus shedding in a specific patient. However, the role of SARS-CoV-2 VL as a biomarker of severity is limited., (© 2020 Wiley Periodicals LLC.)

Purpose: The aim of our study was to assess respiratory function at the time of clinical recovery and 6 weeks after discharge in patients surviving to COVID-19 pneumonia., Methods: Our case series consisted of 13 patients with COVID-19 pneumonia., Results: At the time of clinical recovery, FEV1 (2.07 ± 0.72 L) and FVC (2.25 ± 0.86 L) were lower compared to lower limit of normality (LLN) values (2.56 ± 0.53 L, p = 0.004, and 3.31 ± 0.65 L, p < 0.001, respectively), while FEV1/FVC (0.94 ± 0.07) was higher compared to upper limit of normality (ULN) values (0.89 ± 0.01, p = 0.029). After 6 weeks pulmonary function improved but FVC was still lower than ULN (2.87 ± 0.81, p = 0.014)., Conclusion: These findings suggest that COVID-19 pneumonia may result in clinically relevant alterations in pulmonary function tests, with a mainly restrictive pattern.

Objective: A better understanding of factors influencing breathing weakness in stroke survivors would help in planning rehabilitation therapies. The main objective of this study was to determine whether the location of cerebral infarct is associated with breathing weakness in patients with subacute stroke., Design: Cross-sectional analysis of a prospective cohort., Patients: Consecutive patients admitted to a neurology rehabilitation unit with first-time ischaemic stroke (n?=?170)., Methods: Breathing weakness was defined as >?70% reduction in maximal inspiratory and expiratory pressures (PImax and PEmax, respectively) compared with reference values. Computed tomography and magnetic resonance imaging were used to locate stroke lesions, which were classified as cortical, subcortical, cortico-subcortical, brainstem, or cerebellum. The affected cerebrovascular territory was identified to classify stroke subtype. The association between maximal respiratory pressure and affected brain area was studied using median regression analysis., Results: Breathing weakness was detected in 151 (88.8%) patients. Those with cortical and cortico-subcortical stroke location had the lowest PImax and PEmax values (median 33 cmH2O). This value differed significantly from maximal respiratory pressures of patients with strokes located in the brainstem and the cerebellum, with PImax median differences (?) of 16 cmH2O (95% confidence interval (95% CI) 4.127.9) and 27 cmH2O (95% CI 7.846.2), respectively, and PEmax median differences of 27 cmH2O (95% CI 11.442.7) and 49 cmH2O (95% CI 23.774.3), respectively, both of which remained significant after adjustments., Conclusion: The prevalence of breathing weakness was very high in stroke patients admitted to a neurorehabilitation ward, being more severe in cortical or cortico-subcortical stroke.

Background: Human adenoviruses (HAdVs) are commonly causing respiratory disease. We molecularly genotyped HAdV circulating in Chinese hospitalized children with respiratory infections and summarized the clinical profiles and common inflammatory biomarkers, so as to better determine their associations with disease severity., Method: Children with respiratory single HAdV infection cases that occurred from December 2017 to March 2019 were enrolled for a cross-sectional study. Clinical/laboratory features based on the genotypes of respiratory HAdV infection were reviewed for comparative analysis., Results: A total of 84 patients were enrolled, and HAdV types were identified from 82 patients. Species B (HAdV-7, 44%; HAdV-3, 43%, and HAdV-14, 5%) was the most common, followed by C (HAdV-2, 4% and HAdV-1, 1%) and E (HAdV-4, 1%). Severe HAdV infection and HAdV-7 infection groups were associated with significantly longer duration of fever and hospitalized days, higher morbidity of tachypnea/dyspnea, more pleural effusion, more respiratory rales, more frequently required mechanical ventilation, and significantly higher fatality rate. The elevated procalcitonin (PCT) and C-reactive protein (CRP) levels were significantly associated with severe HAdV infection., Conclusions: HAdV-7 and HAdV-3 were the most common types among children with respiratory adenovirus infection; vaccines against these two genotypes are in urgent need. PCT and CRP are significantly associated with the severity of HAdV infection., Competing Interests: All the authors have reviewed this manuscript and declared no conflict of interest., (Copyright © 2020 Hui Dai et al.)

In the 2013 updated classification of the American Thoracic Society/European Respiratory Society, airway-centered interstitial fibrosis (ACIF) is included as a bronchiolocentric pattern of interstitial pneumonia (IP) among idiopathic IPs. We encountered a case of severe pulmonary hypertension (PH) with chronic IP. The patient initially presented with shortness of breath and often lost consciousness due to PH, and seven years after his first visit, he ultimately died. An autopsy revealed ACIF and usual IP. In particular, the ACIF comprised non-atypical smooth muscle hyperplasia, and pulmonary hypertensive vascular degeneration was detected. This case may represent a new pathological feature of ACIF.

Introduction: Interstitial lung diseases (ILDs) include a wide variety of chronic progressive pulmonary diseases characterized by lung inflammation, fibrosis and hypoxemia and can progress to respiratory failure and even death. ILDs are associated with varying degrees of quality of life impairments in affected people. Studies on the quality of life in patients with ILDs are still limited, and there are few studies with long-term follow-up periods in these patients., Methods: Data from patients who were clinically diagnosed with ILDs in the Respiratory Department, Beijing Chaoyang Hospital, Capital Medical University from January 2017 to February 2018 were collected. Clinical status and HRQoL were assessed at baseline and subsequently at 6- and 12-month intervals with the LCQ, mMRC, HADS, SF-36, and SGRQ. Multivariate linear regression was used to evaluate the determinants of the decline in HRQoL., Results: A total of 139 patients with idiopathic interstitial pneumonia (IIP) and 30 with connective tissue disease-associated ILD (CTD-ILD) were enrolled, 140 of whom completed the follow-up. The mean age was 63.7 years, and 92 patients were men. At baseline, the decline in HRQoL assessed by the SF-36 and SGRQ was significantly associated with the mMRC, LCQ and HADS depression score. In the follow-up, changes in FVC%, DLco%, mMRC and LCQ were significantly associated with changes in HRQoL., Conclusions: HRQoL in both IIP and CTD-ILD patients deteriorates to varying degrees, and the trend suggests that poor HRQoL in these patients is associated with many determinants, primarily dyspnea, cough and depression. Improving HRQoL is the main aim when treating patients living with ILDs., Competing Interests: The authors have declared that no competing interests exist.

Objectives: The ongoing COVID-19 pandemic has caused approximately 2,350,000 infections worldwide and killed more than 160,000 individuals. In Sainte-Anne Hospital (GHU PARIS Psychiatrie & Neuroscience, Paris, France) we have observed a lower incidence of symptomatic forms of COVID-19 among patients than among our clinical staff. This observation led us to hypothesize that psychotropic drugs could have a prophylactic action against SARS-CoV-2 and protect patients from the symptomatic and virulent forms of this infection, since several of these psychotropic drugs have documented antiviral properties. Chlorpromazine (CPZ), a phenothiazine derivative, is also known for its antiviral activity via the inhibition of clathrin-mediated endocytosis. Recentin vitro studies have reported that CPZ exhibits anti-MERS-CoV and anti-SARS-CoV-1 activity., Methods: In this context, the ReCoVery study aims to repurpose CPZ, a molecule with an excellent tolerance profile and a very high biodistribution in the saliva, lungs and brain. We hypothesize that CPZ could reduce the unfavorable course of COVID-19 infection among patients requiring respiratory support without the need for ICU care, and that it could also reduce the contagiousness of SARS-CoV-2. For this purpose, we plan a pilot, multicenter, randomized, single blind, controlled, phase III therapeutic trial (standard treatment vs. CPZ+standard treatment)., Conclusion: This repurposing of CPZ for its anti-SARS-CoV-2 activity could offer an alternative, rapid strategy to alleviate infection severity. This repurposing strategy also avoids numerous developmental and experimental steps, and could save precious time to rapidly establish an anti-COVID-19 therapy with well-known, limited and easily managed side effects., (Copyright © 2020 L'Encéphale, Paris. Published by Elsevier Masson SAS. All rights reserved.)

Respiratory symptoms are common in patients living with serious illness, both in cancer and nonmalignant conditions. Common symptoms include dyspnea (breathlessness), cough, malignant pleural effusions, airway secretions, and hemoptysis. Basic management of respiratory symptoms is within the scope of primary palliative care. There are pharmacologic and nonpharmacologic approaches to treating respiratory symptoms. This article provides clinicians with treatment approaches to these burdensome symptoms., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Introduction: Pulmonary rehabilitation (PR) is known to improve exercise tolerance, mood, and quality of life in patients with chronic respiratory diseases. The aim of this work was to determine whether PR provides long-term benefits in increasing daily life physical activity in patients with chronic sarcoidosis., Methods: This randomized prospective study (registered ClinicalTrials.gov NCT02044939) of 38 patients with stage IV chronic sarcoidosis was performed between 2012 and 2016. Patients were assigned to participate in a 2-month PR program (n=20) or receive counseling (n=18). Assessments were performed at baseline, 2 months (end of the PR program), 6months, and 12months, and included daily life physical activity parameters (measured for 5 consecutive days), exercise tolerance, dyspnea, anxiety, depression, fatigue, and quality of life. The primary outcome was the 12-month change in time spent in activities above an estimated energy expenditure of 2.5metabolic equivalents (METs). Secondary daily life physical activity outcomes included number of steps per day, total daily energy expenditure, and total energy expenditure above 2.5METs., Results: The primary outcome did not differ between the two groups; mean between-group differences were -13.2min (95% confidence interval [CI]: -76.3 to 49.8) at 6 months and -18.1min (95% CI: -55.7 to 19.4) at 12months. Although PR had no effect on secondary daily life physical activity outcomes, it did significantly increase exercise tolerance at 6 and 12 months and decrease the dyspnea score at 6 months and the fatigue score at 12months., Conclusion: This trial failed to demonstrate a beneficial effect of PR on daily life physical activity in sarcoidosis patients, suggesting that long-term behavioral programs may be necessary to complement PR., (Copyright © 2019 SPLF and Elsevier Masson SAS. All rights reserved.)

Purpose: This study aims to examine the effects of nursing interventions based on the Extended Theory of Planned Behavior (ETPB) regarding self-efficacy for exercise (SEE), physical activity (PA), physical function (PF), and quality of life (QOL) in patients with lung cancer who have undergone pulmonary resection., Methods: This quasi-experimental study was conducted between July 2015 and June 2018 in two university-affiliated hospitals. The intervention included pre-operative patient education, goal setting (action and coping planning), and feedback (behavior intention and perceived behavioral control). The intervention group (IG) (n=51) received nursing interventions from the day before surgery to 12 months after lung resection, while the comparison group (CG) (n=36) received usual care. SEE, PA, PF (dyspnea, functional status, and 6-minute walking distance [6MWD]), and QOL were measured before surgery and at one, three, six, and 12 months after surgery. Data were analyzed using the χ² test, Fisher's exact test, Mann-Whitney U test, t-test, and generalized estimation equations (GEE)., Results: There were significant differences between the two groups regarding SEE (χ²=13.53, p =.009), PA (χ²=9.51, p =.049), functional status (χ²=10.55, p =.032), and 6MWD (χ²=15.62, p =.004). Although there were no time or group effects, the QOL mental component (Z=-2.78, p =.005) of the IG was higher than that of the CG one month after surgery. Interventions did not affect dyspnea or the QOL physical component., Conclusion: The intervention of this study was effective in improving SEE, PA, functional status, and 6MWD of lung cancer patients after lung resection. Further extended investigations that utilize ETPB are warranted to confirm these results., Competing Interests: The authors declared no conflict of interest., (© 2020 Korean Society of Nursing Science.)

Purpose: Dyspnea is frequently experienced in advanced cancer patients and is associated with poor prognosis and functional decline. This study used the Edmonton Symptom Assessment System (ESAS) to characterize the relationship between dyspnea and concurrent symptoms experienced by advanced cancer patients., Methods: A prospective database was collected and analyzed to extract patient demographics and ESAS scores. Logistic regression analysis and generalized estimating equations (GEE) identified correlations of other ESAS symptoms in three categories: severity of dyspnea (none, mild, moderate, severe), moderate/severe dyspnea (ESAS ≥ 4), and presence of dyspnea (ESAS ≥ 1), at patients' first visit and over time, respectively., Results: Multivariable analysis revealed drowsiness (p = 0.001), and anxiety (p = 0.01) and appetite loss (p = 0.02) were associated with increased severity of dyspnea at first visit. Over time, tiredness (p = 0.02), drowsiness (p = 0.04), nausea (p = 0.02), and anxiety (p = 0.0006) were more likely to experience increased dyspnea severity. Tiredness (p = 0.0003), depression (p = 0.03), and appetite loss (p = 0.003) were significant for moderate/severe dyspnea at first visit. Over multiple visits, tiredness (p < 0.0001), anxiety (p = 0.0008), and appetite loss (p = 0.0008) had higher probabilities of moderate/severe dyspnea. For the presence of dyspnea at the first visit, anxiety (p = 0.03) and drowsiness (p = 0.002) were significantly correlated with an increased frequency of dyspnea. Over time, anxiety (p < 0.0001) and drowsiness (p < 0.0001) remained significant with the addition of nausea (p = 0.0007)., Conclusions: The highly interactive relationship between dyspnea and other common cancer symptoms necessitates the development of comprehensive symptom assessments and utilization of multimodal management approaches that consider concurrent symptoms for improved identification and treatment of dyspnea.

Background: The optimal prognostic factors in patients with advanced cancer are not known, as a comparison of these is lacking. The aim of the present study was to determine the optimal prognostic factors by comparing validated factors., Materials and Methods: A multicenter, prospective observational cohort study recruited patients over 18 years with advanced cancer. The following were assessed: clinician-predicted survival (CPS), Eastern Cooperative Oncology Group performance status (ECOG-PS), patient reported outcome measures (anorexia, cognitive impairment, dyspnea, global health), metastatic disease, weight loss, modified Glasgow Prognostic Score (mGPS) based on C-reactive protein and albumin, lactate dehydrogenase (LDH), and white (WCC), neutrophil (NC), and lymphocyte cell counts. Survival at 1 and 3 months was assessed using area under the receiver operating curve and logistic regression analysis., Results: Data were available on 478 patients, and the median survival was 4.27 (1.86-7.03) months. On univariate analysis, the following factors predicted death at 1 and 3 months: CPS, ECOG-PS, mGPS, WCC, NC (all p < .001), dyspnea, global health (both p ≤ .001), cognitive impairment, anorexia, LDH (all p < .01), and weight loss ( p < .05). On multivariate analysis ECOG-PS, mGPS, and NC were independent predictors of survival at 1 and 3 months (all p < .01)., Conclusion: The simple combination of ECOG-PS and mGPS is an important novel prognostic framework which can alert clinicians to patients with good performance status who are at increased risk of having a higher symptom burden and dying at 3 months. From the recent literature it is likely that this framework will also be useful in referral for early palliative care with 6-24 months survival., Implications for Practice: This large cohort study examined all validated prognostic factors in a head-to-head comparison and demonstrated the superior prognostic value of the Eastern Cooperative Oncology Group performance status (ECOG-PS)/modified Glasgow Prognostic Score (mGPS) combination over other prognostic factors. This combination is simple, accurate, and also relates to quality of life. It may be useful in identifying patients who may benefit from early referral to palliative care. It is proposed ECOG-PS/mGPS as the new prognostic domain in patients with advanced cancer., Competing Interests: Disclosures of potential conflicts of interest may be found at the end of this article., (© AlphaMed Press 2019.)

Dyspnea is a major symptom of heart failure (HF). Here, we have studied the lung remodeling and airway resistance in HF mice. We demonstrated that aortic banding-induced HF caused a dramatic decrease of lung compliance and an increase of lung airway resistance. The decrease of lung compliance was correlated with the increased lung weight in a linear fashion (γ 2  = 0.824). An HF-induced increase of lung airway resistance and a decrease of lung compliance were almost identical in anesthetized mice and in the isolated lungs from these mice. HF caused profound lung fibrosis in mice with increased lung weight. Moreover, HF patients of NYHA class III-IV showed increased lung density as revealed by high-resolution CT scanning. These data indicate that lung compliance and lung airway resistance may be useful in determining lung remodeling after HF, and lung structure changes may contribute to dyspnea in HF.

An 81-year-old woman with rheumatoid arthritis (RA) who had been treated with bucillamine presented with dyspnea. Computed tomography of the chest showed ground-glass opacities and consolidations in both lungs and honeycombing in both basal lung areas. An elevation of the serum Krebs von den Lungen-6 level and hypoxemia were seen. Lymphocytosis with a decreased CD4/CD8 ratio was seen in the bronchoalveolar lavage fluid. A transbronchial lung biopsy specimen showed organizing pneumonia. Based on a diagnosis of bucillamine-induced pneumonitis (BIP) with RA-associated pre-existing interstitial pneumonia, she was successfully treated with the cessation of bucillamine and systemic corticosteroid therapy. The risk factors and prognosis of BIP are discussed.

Objective: Pleuropulmonary blastoma (PPB) is a rare malignant tumor in childhood that is highly invasive and has poor prognosis. We retrospectively analyzed patients with PPB who died within 30 days in hopes of providing a basis for improving diagnosis and treatment., Methods: We retrospectively reviewed six children with PPB who died within 30 days of admission at our hospital from January 2004 to March 2018, including their clinical features, pathological diagnosis, course of treatment, and major causes of death., Results: Six patients (two female, four male; median age, 38 months) were included. All patients presented with respiratory symptoms. Chest imaging showed that all tumors had diameters greater than 10 cm, with varying degrees of serous effusion. Four patients underwent ultrasound-guided fine-needle aspiration (FNA), one patient underwent exploratory thoracotomy, and one underwent partial tumor resection. Five cases were type III, and another was type II. Four patients developed adverse events while waiting for pathological results after FNA, and four patients were treated with chemotherapy but their tumors failed to decrease in size one to two weeks later. The median hospitalization to death time was 17 days (range, 5-24 days)., Conclusions: PPB often presents with respiratory symptoms that rapidly develop into respiratory distress. The rapid tumor enlargement contributes to the disease's progression. Chemoreduction in such tumors is not obviously effective, and the mortality rate is high. The main causes of death were respiratory failure and sepsis. Further clinical studies will be required to evaluate the role of initial biopsy compared with upfront total excision., (© 2019 Wiley Periodicals, Inc.)

Competing Interests: None

A 64-year-old manpresented with non-productive cough and dyspnoea and was evaluated and diagnosed to have a left lung mass on CT of the chest. A transthoracic needle biopsy under CT guidance revealed necrotic tissue on histopathology and was inconclusive. Positron emission tomography scan revealed a fluoro-deoxyglucose-avid left lung mass with a left upper lobe luminal cut-off. A flexible video bronchoscopy was performed and revealed left upper lobe complete obstruction with an endoluminal plug which was removed in piecemeal fashion, and deeper biopsies were taken from the lingula. Histopathology revealed underlying adenocarcinoma colonised by aspergillosis. This case serves to remind us of the possibility of missing underlying malignancy when taking superficial biopsies of clearly visualised endobronchial necrotic tissue and the need for debulking it to a reasonable extent and to take deeper biopsies in order to not miss a possible underlying malignancy., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

A 63-year-old man presented with persistent cough and progressive dyspnea. Computed tomography showed irregular pleural thickening and fibrotic changes with volume loss in the upper lobes, and subtle reticulation in the lower lobes. Pleuroparenchymal fibroelastosis (PPFE) was diagnosed based on the findings of a surgical lung biopsy. Bronchiolar lesions, including proliferative bronchiolitis, constrictive bronchiolitis obliterans, and peribronchiolar metaplasia were evident on pathology. A usual interstitial pneumonia (UIP) pattern was also observed in the lower lobes. Three weeks after the biopsy, an acute exacerbation occurred. We herein describe a rare case of idiopathic PPFE with various bronchiolar lesions and a UIP pattern in which an acute exacerbation developed.

Pulmonary tumor thrombotic microangiopathy (PTTM) is a complication characterized by dyspnea, pulmonary hypertension, and occasionally sudden death. We encountered a man who developed PTTM and had an inhalation history of chemical herbicides and abnormal findings on chest computed tomography, mimicking chemical inhalation lung injury. He was diagnosed with PTTM with adenocarcinoma by a transbronchial lung biopsy and received chemotherapy and anticoagulant therapy. He survived for one month. An autopsy revealed primary gastric cancer with PTTM that can have a presentation similar to diffuse pulmonary diseases, including chemical inhalation lung injury. The examination of a biopsy specimen is crucial in such patients.

Pneumonia is frequently complicated by occurrence of acute respiratory distress syndrome (ARDS), consequently to dysregulated inflammatory response. However, mechanisms driving this dysregulation are poorly understood. To address this, "unconventional T-cells (UTC)" -γδT, NKT and MAIT cells- appear to be relevant targets due to their key role in orchestrating anti-microbial immune response in the lung. Thus, using an experimental and translational approach, we test the hypothesis that a tight regulation of UTC is mandatory to fine-tune host response, and, subsequently to prevent emergence of an aberrant response leading to excessive tissue damages, and eventually, ARDS., (Copyright © 2019 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Objectives: Our study aimed to analyze the differences in intra-amniotic pressures in patients with polyhydramnios with and without symptoms., Study Design: We recruited patients with pregnancies in which amnioreduction was performed for polyhydramnios in the Department of Fetal-Maternal Medicine at Nagara Medical Center between April 2017 and August 2018. Amnioreduction was performed for severe polyhydramnios with maternal symptoms [symptomatic group] or polyhydramnios without maternal symptoms [asymptomatic group] such as abdominal distension, dyspnea, or threatened premature labor. We measured the intra-amniotic pressure after every 200 ml volume reduction during the amnioreduction., Results: A total of 27 patients who underwent amnioreduction were classified into symptomatic (66.7%, 18/27) and asymptomatic (33.3%, 9/27) groups. Gestational age, amniotic fluid index at the time of amnioreduction, and the volume of amniotic fluid removed were not significantly different between the symptomatic and asymptomatic groups [median 32.4 weeks vs. 33.1 weeks, median 38.0 cm vs. 39.0 cm, and median 1500 ml vs. 2500 ml, respectively]. However; the intra-amniotic pressure before amnioreduction was significantly higher in the symptomatic group than in the asymptomatic group [median 15.0 mmHg (range, 10-27) vs. 10.0 mmHg (range, 6.0-13); p < 0.005]. After amnioreduction, these pressures decreased significantly to median 9.0 mmHg (range, 5.0-13) (p < 0.001) in the symptomatic and 7.0 mmHg (range, 4.0-11) (p < 0.05) in the asymptomatic group. The median intra-amniotic pressure gradually decreased and reached a plateau during the amnioreductions in both groups., Conclusions: With polyhydramnios, the intra-amniotic pressure was significantly higher in the symptomatic group than in the asymptomatic group. Therefore, uterine pressure tolerance might vary according to the individual. In addition, intra-amniotic pressure monitoring might enhance the safety during amnioreduction procedures to avoid drastic and potentially harmful pressure changes., (Copyright © 2019 Elsevier B.V. All rights reserved.)

A 38-year-old woman developed a spontaneous right-sided tension pneumothorax during light aircraft travel. The aircraft was diverted to a regional centre, where emergent needle thoracostomy and chest tube insertion were performed. History suggested that this was the second episode of pneumothorax, with an untreated event with similar symptomatology during air travel 1 year ago. She was taken for surgical intervention. Intraoperative findings were of a large right middle lobe cyst of uncertain origin; the procedure was subsequently aborted. A CT chest demonstrated a large multiseptated air-filled pulmonary cystic lesion. Inpatient stay was notable for persistent right pneumothorax with interval cyst rupture. A right middle lobectomy was subsequently performed with histopathology showing a benign epithelioid bronchogenic cyst. Recovery was unremarkable with no residual pneumothorax or further episodes at 2 months postoperatively. Preventative excision of air-filled pulmonary abnormalities should be considered prior to air travel., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Purpose: Chemoradiation (CRT) is an integral treatment modality for patients with locally advanced lung cancer. It has been hypothesized that current use of an angiotensin-converting enzyme inhibitor during CRT may be protective for treatment-related lung damage and pneumonitis., Methods and Materials: We conducted a pilot, double-blind, placebo-controlled, randomized trial. Study-eligible patients receiving curative thoracic radiation therapy (RT) were randomly assigned to 20 mg of lisinopril or placebo once daily during and up to 3 months after RT. All patients received concurrent chemotherapy. The primary endpoint was adverse event profiling. Multiple patient-reported outcome (PRO) surveys, including the Lung Cancer Symptom Scale, Function Assessment of Cancer Therapy-Lung, and the European Organisation for Research and Treatment of Cancer Lung Cancer Questionnaire, were applied with a symptom experience questionnaire. Exploratory comparative statistics were used to detect differences between arms with χ 2 and Kruskal-Wallis testing., Results: Five institutions enrolled 23 patients. However, accrual was less than expected. Eleven and 12 patients were in the placebo and lisinopril arms, respectively (mean age, 63.5 years; male, 62%). Baseline characteristics were balanced. Eighteen patients (86%) were former or current smokers. The primary endpoint was met; neither arm had grade 3 or higher hypotension, acute kidney injury, allergic reaction (medication-induced cough), or anaphylaxis (medication-related angioedema). Few PRO measures suggested that compared with the placebo arm, patients receiving lisinopril had less cough, less shortness of breath, fewer symptoms from lung cancer, less dyspnea with both walking and climbing stairs, and better overall quality of life (for all, P < .05)., Conclusions: Although underpowered because of low accrual, our results suggest that there was a clinical signal for safety-and possibly beneficial by limited PRO measures-in concurrently administering lisinopril during thoracic CRT to mitigate or prevent RT-induced pulmonary distress. Our results showed that a definitive, larger-scale, randomized phase 3 trial is needed in the future., (Copyright © 2018. Published by Elsevier Inc.)

Rationale: Pulmonary embolism is a potentially devastating medical condition. Although deep-vein thrombosis is the most common etiology, a nonthrombotic pulmonary embolism is not uncommon., Patient Concerns: A 45-year-old woman was admitted to our hospital for progressive effort dyspnea for 3 weeks., Diagnosis: Echocardiography revealed a mobile mass extending from the right atrium to the bilateral proximal pulmonary artery. As the mass was no response to thrombotic therapy, intravenous leiomyomatosis was suspected. Computed tomography (CT) revealed a hypo-attenuated tumor extending from the ovarian vein to the pulmonary artery., Interventions: A 1-stage operation for the surgical removal of the tumor, right salpingo-oophorectomy and subtotal hysterectomy were performed. Surgical specimen was identified as uterine leiomyomatosis without malignant transformation., Outcomes: The patient was followed- up for 2 years and subsequent CT did not reveal any notable lesions., Lessons: Nonthrombotic pulmonary embolism is a potentially life-threatening complication. This case indicated intravenous leiomyomatosis should be considered in the differential diagnosis for certain cases. Complete surgical excision was the only curative treatment.

A 64-year-old man was admitted to our hospital for purpuric rash, joint pain, and a fever. He had earlier undergone a follow-up examination for interstitial lung disease. At the current visit, the diagnosis was immunoglobulin A (IgA) vasculitis, based on skin and renal biopsy findings. He developed sudden breathlessness and hemoptysis. Chest computed tomography revealed ground glass opacity in the right lower lung fields, suggesting pulmonary hemorrhaging associated with IgA vasculitis. Despite steroid and cyclophosphamide therapy, and plasma exchange, he died 52 days after admission. Early aggressive therapies may be recommended for old patients with IgA vasculitis who have an additional comorbidities.

Dyspnea has been reported to be a main contributor to exercise avoidance in asthma. While traditional markers of ventilation do not explain the heightened dyspnea during exercise in patients with asthma, this study proposed that exertional dyspnea in asthma was due to high-operating lung volumes, which may be improved with a short-acting β2-agonist. On two separate days, 16 patients with asthma and 16 controls completed a lung function test and incremental exercise tests to exhaustion. On one of the days (order randomized), 400 µg salbutamol was administered before exercise. Inspiratory capacity (IC), inspiratory reserve volume (IRV), and dyspnea (modified Borg scale) were evaluated throughout exercise. Compared with controls, patients with asthma reported greater dyspnea at the same absolute submaximal workloads. Furthermore, patients with asthma demonstrated altered breathing responses to exercise, characterized by reduced IC and IRV throughout exercise compared with controls. The reduced IRV was associated with increased dyspnea in patients with asthma. Salbutamol did not affect dyspnea or operating lung volumes in either group. The increased perception of dyspnea during incremental exercise in patients with asthma appears to be secondary to a reduction in IRV, which is unaffected by an inhaled β2-agonist. NEW & NOTEWORTHY Increased exertional dyspnea in asthma appears to be due to high operating lung volumes and is not affected by salbutamol.