Combined pulmonary fibrosis and emphysema.

PURPOSE OF REVIEW: Combined pulmonary fibrosis and emphysema (CPFE) is a syndrome characterized by upper lobe emphysema with lower lobe fibrosis. We aim to bring some clarity about its definition, nature, pathophysiology, and clinical implications. RECENT FINDINGS: Although multiple genetic and molecular pathways have been implicated in the development of CPFE, smoking is considered the most prevalent risk factor. CPFE is most prevalent in middle-aged men with more than 40 pack-years of smoking and can be seen in about 8% of all chronic obstructive pulmonary disease (COPD) patients. Given its nature, it is a radiological diagnosis, better defined by computed tomography (CT). Spirometry can be normal despite severe disease or can have restrictive or obstructive patterns, but the diffusing capacity of the lungs (DLCO) is consistently low regardless of the spirometry pattern. The disease is progressive, with high occurrences of lung cancer and pulmonary hypertension, complications that limit survival. Unfortunately, there is no treatment found to be beneficial other than supportive care and guideline-directed medical therapy. SUMMARY: CPFE is best described as a clinical and radiological syndrome where smokers are particularly at greater risk. Although simplistic, the earliest definition based chiefly on radiographic findings can identify a patient population with similar physiology. The most recent consensus proposes the definition based on mainly radiological findings with impaired gas exchange.

Asbestos-associated pulmonary disease.

PURPOSE OF REVIEW: Exposure to asbestos can cause both benign and malignant, pulmonary and pleural diseases. In the current era of low asbestos exposure, it is critical to be aware of complications from asbestos exposure; as they often arise after decades of exposure, asbestos-related pulmonary complications include asbestosis, pleural plaques, diffuse pleural thickening, benign asbestos-related pleural effusions and malignant pleural mesothelioma. RECENT FINDINGS: Multiple recent studies are featured in this review, including a study evaluating imaging characteristics of asbestos with other fibrotic lung diseases, a study that quantified pleural plaques on computed tomography imaging and its impact on pulmonary function, a study that examined the risk of lung cancer with pleural plaques among two large cohorts and a review of nonasbestos causes of malignant mesothelioma. SUMMARY: Asbestos-related pulmonary and pleural diseases continue to cause significant morbidity and mortality. This review summarizes the current advances in this field and highlights areas that need additional research.

Influence of Iron Deficiency on Clinical and Haemodynamic Parameters in Pulmonary Arterial Hypertension Cohorts.

BACKGROUND: Iron deficiency (Fedef) has been shown to be common in patients with group 1 or pulmonary arterial hypertension (PAH). Several studies have shown a negative impact of Fedef on clinical and haemodynamic parameters of the disease, but data from individual studies have not been strong enough to lead to incorporation of the finding of Fedef into prognostic or therapeutic algorithms. The goal of this meta-analysis was to combine data from available studies to better define any associations between Fedef and established variables of prognostic importance in PAH. METHODS: A literature search identified nine studies with extractable data relevant to the study questions. The impact of Fedef upon the following parameters was evaluated: 6-minute walk distance (6MWD), WHO-functional class, N-terminal pro-brain natriuretic peptide (NT-proBNP) levels, echocardiography, and findings from right heart catheterisation (RHC). Pooled results were reported as mean difference or risk difference with 95% confidence intervals utilising a random effects modeling approach. RESULTS: Fedef in the PAH population was common (47% of cases) and was associated with cardiovascular dysfunction (lower tricuspid annular plane systolic excursion [TAPSE], elevated NT-proBNP, and lower mixed venous oxygen saturation) and with reduction in functional capacity (lower 6MWD and higher functional class). CONCLUSION: This meta-analysis strengthens the relationships between Fedef and several markers of poor outcome in PAH. Fedef in patients with PAH warrants further scrutiny and merits consideration as a cause of clinical deterioration. Even though causation and longitudinal relationships between Fedef and PAH could not be identified, effect of Fedef on factors that affect disease prognosis is noteworthy and worthy of more focussed studies.

A 72-Year-Old Woman With Dyspnea, Multiple Pulmonary Nodules, and Mediastinal Lymphadenopathy.

CASE PRESENTATION: A 72-year-old woman presented to our institution with gradually worsening shortness of breath and bilateral lower extremity edema of 3 weeks' duration. She had associated complaints of cough and intermittent hemoptysis. Her medical history was significant for hypertension and hypothyroidism. She was a former cigarette smoker with a 35 pack-year smoking history. She had no recent travel history and had a pet dog at home. Six months before the current hospitalization, evaluation for cough had revealed mediastinal lymphadenopathy at an outside institution. She underwent evaluation with an endobrachial ultrasound procedure at an outside facility 8 weeks before the current admission. The procedure demonstrated both acute and chronic inflammation, with one specimen showing few atypical cells on cytopathology and no growth on bacterial, fungal, and mycobacterial cultures. She was treated empirically with oral steroids for presumed sarcoidosis. However, this did not result in clinical benefit, and because of progressive symptoms, she presented to our institution.

A 34-Year-Old Man With Lightheadedness and Dyspnea.

CASE PRESENTATION: A 34-year-old man presented to our institution with lightheadedness and dyspnea on exertion. His medical history included chronic pancreatitis, juvenile rheumatoid arthritis (JRA), gastroesophageal reflux disease, hypertension, lumbar degenerative disc disease, seizure disorder, anterior uveitis, and multiple vertebral fractures. In addition, he was a cigarette smoker with a 10-pack-year smoking history.

Treatment of COVID-19 Patients with Two Units of Convalescent Plasma in a Resource-Constrained State.

IMPORTANCE: Many therapies are used to treat COVID-19, the disease caused by the virus SARS-CoV-2, including convalescent plasma. The clinical utility of using 2 units of convalescent plasma for COVID-19 hospitalized patients is not fully understood. OBJECTIVE: Many therapies are used to treat COVID-19, the disease caused by the virus SARS-CoV-2, including convalescent plasma. The clinical utility of using 2 units of convalescent plasma for COVID-19 hospitalized patients is not fully understood. Our study aims to determine the safety and efficacy of treating hospitalized COVID-19 patients with 2 units of COVID-19 convalescent plasma (CCP). METHOD: This was a retrospective study of Arkansas patients treated with CCP using the (US) Food and Drug Administration (FDA) emergency Investigational New Drug (eIND) mechanism from April 9, 2020, through August 9, 2020. It was a multicenter, statewide study in a low-resource setting, which are areas that lack funding for health care cost coverage on various levels including individual, family, or social. Adult patients (n = 165, volunteer sample) in Arkansas who were hospitalized with severe or life-threatening acute COVID-19 disease as defined by the FDA criteria were transfused with 2 units of CCP (250 mL/unit) using the FDA eIND mechanism. The primary outcome was 7- and 30-day mortality after the second unit of CCP. RESULTS: Unadjusted mortality was 12.1% at 7 days and 23.0% at 30 days. The unadjusted mortality was reduced to 7.7% if the first CCP unit was transfused on the date of diagnosis, 8.7% if transfused within 3 days of diagnosis, and 32.0% if transfused at or after 4 or more days of diagnosis. The risk of death was higher in patients that received low, negative, or missing titer CCP units in comparison to those that received higher titer units. CONCLUSION: The provision of 2 units of CCP was associated with a reduction in mortality in patients treated with high titer units within 3 days of COVID-19 diagnosis. Given the results, CCP is a viable, low-cost therapy in resource-constrained states and countries.

Thrombotic thrombocytopenic purpura (TTP) after COVID-19 vaccination: A systematic review of reported cases.

INTRODUCTION: With the advent of COVID-19 vaccines, hospitalization rates and progression to severe COVID-19 disease have reduced drastically. Most of the adverse events reported by the vaccine recipients were minor. However, autoimmune hematological complications such as vaccine-induced immune thrombotic thrombocytopenia (VITT), immune thrombocytopenic purpura (ITP) and TTP have also been reported post-COVID-19 vaccination. Given this, we sought to reflect on the existing cases of TTP, whether de novo or relapsing, reported after COVID-19 vaccination to further gain insight into any association, if present, and outcomes. METHODS: We searched PubMed, Embase, and Ebsco databases for published individual case reports on the occurrence or relapse of TTP after receiving any COVID-19 vaccine. A total of 23 articles (27 patients) were included in this qualitative analysis. RESULTS: The mean age for the patients who developed de novo TTP post-COVID-19 vaccination was 51.3 years. TTP episodes were seen mostly after BNT162b2 vaccine, followed by mRNA-1273 vaccine. All patients with immune TTP except one received plasma exchange (PLEX) and steroids. One patient passed away after two days of hospitalization, likely due to a sudden cardiovascular event. CONCLUSION: Our review underscores the importance of in-depth anamnesis before vaccination and outlines characteristics of predisposed individuals. Evaluation of post-vaccine thrombocytopenia must include the possibility of TTP given the associated fatality with this condition.

A 44-Year-Old Woman With a 10-Year History of Dyspnea and Pulmonary Nodules.

CASE PRESENTATION: A 44-year-old woman was referred for evaluation of dyspnea on exertion and multiple nodular opacities on a chest CT scan. She had a medical history of autoimmune encephalitis, diabetes mellitus, hypertension, migraines, and allergic rhinitis. Ten years earlier, the patient was admitted to an outside institution with symptoms of shortness of breath. She was found to have multiple pulmonary nodules and was diagnosed empirically with and treated for sarcoidosis. She was told that her pulmonary nodules had improved on follow up. However, she continued to have symptoms of dyspnea. Due to progressive symptoms of shortness of breath, she was referred to pulmonology. She reported a weight gain of 80 pounds over the last year. She denied fever, chills, hemoptysis, night sweats, joint swelling, or skin rash. She is a former cigarette smoker with a 15 pack-year smoking history, quit smoking in 2005. She denied alcohol or drug use. She resided in Arkansas and Texas over the past decade. She previously worked as a teacher and is currently unemployed. She had no other relevant exposures. She denied a family history of autoimmune diseases or malignancies.

A 41-Year-Old Man Presenting With Shortness of Breath, Nausea, Vomiting, and Diarrhea.

CASE PRESENTATION: A 41-year-old man presented to our institution with shortness of breath for 1 day, and nausea, vomiting, and diarrhea for 10 days. He has a medical history of gastroesophageal reflux disease, migraines, and anxiety. He is a 10 pack-year former cigarette smoker and an active vaper. He quit smoking cigarettes in 2013 but reports vaping flavored nicotine from 2013 to 2018. Since 2018, he has been vaping tetrahydrocannabinol products of different flavors and brands. A few weeks prior to admission, the subject had changed the brand of his vaping product to "Cookies High Flyers" with a "Biscotti" flavor. The new product contains 1,000 mg (883 mg tetrahydrocannabinol and 117 mg cannabidiol) in a 0.04 oz cartridge. He vapes five times a day, taking two to three puffs every time. He had recently traveled to Texas and had a sick contact with his 18-month-old daughter. She recently recovered from a diarrheal illness of presumed viral origin. Prior to admission, the subject tested negative for influenza and completed outpatient antibiotic treatment, with no improvement.

Vaping epidemic in US teens: problem and solutions.

PURPOSE OF REVIEW: Over the last decade, vaping has emerged into an epidemic of alarming proportions among US teens. This review evaluates the factors leading to the rise of vaping, reasons for its striking popularity among US teens, health consequences of vaping, and measures to mitigate the vaping epidemic. RECENT FINDINGS: Contemporary research highlights the continued rise of vaping amongst US teens and explains the reasons for its popularity, such as the variety of devices, a wide range of available flavors, youth-targeted advertisements, and lack of awareness of adverse consequences from vaping. This publication highlights current research findings of health consequences of vaping, including a discussion of EVALI (e-cigarette or vaping product use-associated lung injury) and provides an update on strategies to curtail the vaping epidemic. SUMMARY: Vaping has risen to epidemic proportions amongst US teens. This poses a clear and present danger to teens' health with adverse effects ranging from acute lung injury to long-term addiction. This article summarizes key research findings that explain the reasons for the epidemic, health consequences of vaping, and provides an overview of efforts to mitigate the vaping threat to US teens.

Undiagnosed Acquired Hemophilia A: Presenting as Recurrent Gastrointestinal Bleeding.

Acute gastrointestinal bleeding (GIB) is a frequently encountered medical emergency and it can be life-threatening depending on the etiology and the clinical condition of the patient. The most common causes of GIB are peptic ulcer disease, aspirin-induced gastritis, variceal hemorrhage, esophagitis, neoplasms like gastric cancer. Acquired hemophilia causing acute gastrointestinal bleed is extremely rare and only a few cases are reported worldwide. Acquired hemophilia A (AHA) is a rare disorder caused by the production of autoantibodies that inactivates clotting factor VIII. We present a case of upper gastrointestinal bleed due to AHA which was undiagnosed for two years. A 74-year-old patient with a history of myasthenia gravis, presented with anemia, and GIB. She underwent multiple endoscopies without a clear bleeding source. Coagulation studies showed isolated activated partial thromboplastin time prolongation which was not corrected by mixing study. Factor VIII activity was low and Bethesda titer showed elevated inhibitor levels. Factor Eight Bypassing Agent, recombinant factor VIIa, and steroids were given to control bleeding. Her clinical condition worsened, and she passed away. Elderly patients presenting with an undiagnosed source of GIBs, inconclusive endoscopic studies should be evaluated for acquired coagulopathies, especially in those with a history of autoimmune diseases and malignancies. Prompt diagnosis and treatment are warranted as it carries a high mortality. Part of the case presentation was presented as an abstract at a regional conference.

Prevalence of Chronic Obstructive Pulmonary Disease in Patients Diagnosed with Cerebrovascular Accidents and Its Effect on Health Care Utilization: A Cross-sectional Study.

BACKGROUND: To assess prevalence and to determine the impact of chronic obstructive pulmonary disease (COPD) on health care utilization in patients with cerebrovascular accident (CVA). METHODS: We performed retrospective analysis of data from 12,102 patients who had diagnosis of CVA from 2014 to 2019 at tertiary medical center. We calculated the prevalence of COPD among patients with diagnosis of CVA. We performed unadjusted, covariate adjusted, and propensity-matched analysis to evaluate differences in health care utilization in patients with CVA and COPD compared to patients with CVA without COPD. RESULTS: 12,102 patients were diagnosed with CVA episodes. The prevalence of COPD among CVA patients was 7.65 % (95%CI: 7.18-8.13). The unadjusted and covariate adjusted analysis demonstrated that the average number of hospitalizations among CVA patients with a diagnosis of COPD was significantly higher than CVA patients without COPD. After adjusting for modifiable and nonmodifiable confounders, CVA patients diagnosed with COPD have on average 1 more hospitalization (1.21; 95%CI: 1.12-1.30) than those who are not diagnosed with COPD. Subsequent analysis based on propensity-matched data suggests that CVA patients diagnosed with COPD have on average approximately 1 more hospitalization (1.44; 95% CI: 1.31-1.58) than CVA patients without COPD. CONCLUSIONS: Our study suggests significant prevalence of COPD among CVA patients. The presence of COPD as a comorbidity resulted in patients with COPD and CVA having increased number of hospitalizations compared to CVA patients without COPD.

Asthma-COPD overlap: review of diagnosis and management.

PURPOSE OF REVIEW: Asthma and chronic obstructive pulmonary disease are both commonly encountered respiratory conditions. The term asthma--COPD overlap (ACO) has been used to identify patients presenting with features of both conditions. Controversy exists regarding its definition, approach to diagnosis and management. In this publication, recent evidence has been reviewed that provides insight into diagnosis and management of this condition. RECENT FINDINGS: Previously, multiple criteria were used to define Asthma--COPD overlap. In this publication, the most recent guidelines to identify this condition have been reviewed. This publication provides a summary of the recent evidence with regard to the role of various diagnostic modalities including the use of biomarkers, such as exhaled nitric oxide, serum IgE and provides updated evidence on available treatment choices for this condition. SUMMARY: ACO is a commonly encountered clinical condition with patients experiencing frequent exacerbations and resulting in increased healthcare resource utilization. Recent interest in ACO has led to development of a framework towards diagnosis and management of this condition. Therapeutic choices for ACO range from bronchodilator therapy to immunomodulatory therapy, highlighting the heterogeneity of this condition. Additional research is required to improve understanding of pathogenesis and improve outcomes in ACO.

Are depressive symptoms related to physical inactivity in chronic obstructive pulmonary disease?

PURPOSE: Patients with chronic obstructive pulmonary disease (COPD) are physically inactive, and this predicts poor outcome. Factors influencing activity levels in COPD patients are poorly understood. Depression is common in COPD patients and may influence activity. Accordingly, in this pilot study, we evaluated the relationship between depressive symptoms and activity in clinically stable COPD (forced expiratory volume in 1 second [FEV(1)]/forced vital capacity < 0.70, FEV(1) < 80%). An additional inclusion criterion was the Hospital Anxiety and Depression Scale depression score, which had to be ≤7 or ≥10, representing low and high levels of depressive symptoms, respectively. METHODS: Sixteen patients with high depression scores (DEPR) and 20 with low depression scores (non-DEPR), were studied. Physical activity was measured over 7 consecutive days, using the SenseWear armband (BodyMedia Inc, Pittsburgh, PA) worn on the arm. RESULTS: The mean age was 69 ± 9 years and the FEV(1) was 49 ± 16%. The Hospital Anxiety and Depression Scale depression scores in DEPR and non-DEPR patients were 12.0 ± 2.3 and 3.7 ± 2.0, respectively. There were no significant between-group differences in age, gender, body mass index, FEV(1), supplemental oxygen requirement, and walk distance. DEPR and non-DEPR patients did not differ in estimated steps per day (3490 ± 2020 vs 3634 ± 2000; P = .83) or minutes per day, with activity >3 metabolic equivalents (11 ± 7 vs 13 ± 9; P = .42). Steps correlated with the FEV(1) (r = 0.49; P = .002) and the 6-minute walk distance (r = 0.69; P = .0001). CONCLUSIONS: We were unable to demonstrate a relationship between depressive symptoms and directly measured physical activity level in patients with COPD.