Diffuse Pulmonary Ossification as a Rare Cause of Interstitial Lung Disease

Diffuse pulmonary ossification (DPO) is a rare form of interstitial lung disease. The present article describes a case of DPO in an elderly man who presented with progressive dyspnea on exertion and an isolated reduction in diffusing capacity for carbon monoxide. DPO may occur as sequelae of mitral stenosis, left heart failure, idiopathic pulmonary fibrosis, recurrent aspiration pneumonia, solid organ transplant, adult respiratory distress syndrome or may arise idiopathically. In the absence of other findings of interstitial lung disease, a lung biopsy is unlikely to be helpful in the management of these patients

Pulmonary actinomycosis in a male patient with a tracheal bronchus

BACKGROUND: Tracheal bronchus is a congenital malformation comprising an abnormal bronchus originating from the trachea or other bronchus. This malformation has been associated with recurrent pneumonia in children, but is rarely associated with infection in adults. Actinomyces species are rare causes of necrotizing pneumonias that often masquerade as malignancy, lung abscesses and tuberculosis

Recurrent spontaneous pneumothoraces and bullous emphysema. A novel mutation causing Birt-Hogg-Dube syndrome

AbstractBirt-Hogg-Dube syndrome (BHDS) is a rare form of classically cystic lung disease that may present with spontaneous pneumothorax. The associated skin manifestations (fibrofolliculomas) are not always present. This article describes a case of spontaneous pneumothorax secondary to bullous emphysema in an otherwise healthy gentleman caused by a novel mutation in the folliculin (FLCN) gene

McArdle's Disease Presenting as Unexplained Dyspnea in a Young Woman

McArdle's disease is a rare, inherited deficiency of myophosphorylase, an enzyme required for the utilization of glycogen. Patients with myophosphorylase deficiency classically present with exercise intolerance, leg pain and muscle fatigue. The case of a young woman with exertional dyspnea and leg cramps is described. Exercise testing confirmed the presence of exercise intolerance and demonstrated an accelerated heart rate response, despite the absence of an anaerobic threshold and a respiratory exchange ratio of less than 1.0. Subsequent ischemic forearm testing and muscle biopsy confirmed the diagnosis of myophosphorylase deficiency. Evaluation of lung mechanics with esophageal pressure measurements demonstrated the presence of respiratory muscle weakness and early fatiguability, suggesting that the patient's dyspnea might have been attributable to an increased respiratory effort. Dyspnea is not a classic symptom associated with myophosphorylase deficiency, although subclinical respiratory muscle impairment may be present. No previous studies have evaluated respiratory muscle function during exercise in patients with myophosphorylase deficiency

Effects of oxygen on exercise duration in chronic obstructive pulmonary disease patients before and after pulmonary rehabilitation

BACKGROUND: Supplemental oxygen therapy has been shown to improve exercise performance in patients with chronic obstructive pulmonary disease (COPD). It is unknown whether the magnitude of this benefit would be affected by participation in a pulmonary rehabilitation program

The Impact of Multidisciplinary Discussion (MDD) in the Diagnosis and Management of Fibrotic Interstitial Lung Diseases

Accurate diagnosis of interstitial lung disease (ILD) is crucial for management and prognosis but can be challenging even for experienced clinicians. Expert multidisciplinary discussion (MDD) is considered the reference standard for ILD diagnosis; however, there remain concerns regarding lack of validation studies and relative limited information on the impact of MDD in real-life clinical practice. The goal of this study was to assess the effect of MDD in providing a specific ILD diagnosis, changing the diagnosis provided upon referral, and to determine how often and in which way MDD altered management. Material and Methods. Retrospective observational study in an ILD referral tertiary academic center. MDD diagnoses were categorized as specific, provisional, and unclassifiable ILD. Pre-MDD and MDD diagnoses were compared for change in diagnosis and concordance rates for specific diagnoses. Relevant change in management including initiation or change in pharmacological treatment, referral to surgical biopsy, and nonpharmacological management were recorded. Results. 126 cases were included (79M, 47F, 36–93 years, mean 70 y). Specific MDD diagnosis was provided in 62% (78/126); 12% (15/126) had provisional diagnosis, and 21% (27/126) was unclassifiable. Overall agreement for specific pre-MDD and MDD diagnosis was 41% (52/126) and 80% for idiopathic pulmonary fibrosis (IPF) diagnosis. MDD altered diagnosis in 37% (47/126) and changed management in 39% (50/126). Amongst concordant diagnoses, management was altered in 46% (24/52). In summary, MDD provided a specific diagnosis discordant with pre-MDD diagnosis in a significant proportion of cases and was particularly valuable in the diagnosis of non-IPF ILD. MDD often altered management and had relevant impact on management even in cases with concordant pre-MDD diagnosis

Exercise responses and mental health symptoms in COVID-19 survivors with dyspnoea

Objectives Dyspnoea is a common persistent symptom post-coronavirus disease 2019 (COVID-19) illness. However, the mechanisms underlying dyspnoea in the post-COVID-19 syndrome remain unclear. The aim of our study was to examine dyspnoea quality and intensity, burden of mental health symptoms, and differences in exercise responses in people with and without persistent dyspnoea following COVID-19. Methods 49 participants with mild-to-critical COVID-19 were included in this cross-sectional study 4 months after acute illness. Between-group comparisons were made in those with and without persistent dyspnoea (defined as modified Medical Research Council dyspnoea score ≥1). Participants completed standardised dyspnoea and mental health symptom questionnaires, pulmonary function tests, and incremental cardiopulmonary exercise testing. Results Exertional dyspnoea intensity and unpleasantness were increased in the dyspnoea group. The dyspnoea group described dyspnoea qualities of suffocating and tightness at peak exercise (p<0.05). Ventilatory equivalent for carbon dioxide (VʹE/VʹCO2) nadir was higher (32±5 versus 28±3, p<0.001) and anaerobic threshold was lower (41±12 versus 49±11% predicted maximum oxygen uptake, p=0.04) in the dyspnoea group, indicating ventilatory inefficiency and deconditioning in this group. The dyspnoea group experienced greater symptoms of anxiety, depression and post-traumatic stress (all p<0.05). A subset of participants demonstrated gas-exchange and breathing pattern abnormalities suggestive of dysfunctional breathing. Conclusions People with persistent dyspnoea following COVID-19 experience a specific dyspnoea quality phenotype. Dyspnoea post-COVID-19 is related to abnormal pulmonary gas exchange and deconditioning and is linked to increased symptoms of anxiety, depression and post-traumatic stress