Digital Health Technology and Telemedicine-Based Hospital and Home Programs in Pulmonary Medicine During the COVID-19 Pandemic.

BACKGROUND: The current coronavirus disease 2019 (COVID-19) pandemic has caused a significant strain on medical resources throughout the world. A major shift to telemedicine and mobile health technologies has now taken on an immediate urgency. Newly developed devices designed for home use have facilitated remote monitoring of various physiologic parameters relevant to pulmonary diseases. These devices have also enabled home-based pulmonary rehabilitation programs. In addition, telemedicine and home care services have been leveraged to rapidly develop acute care hospital-at-home programs for the treatment of mild-to-moderate COVID-19 illness. AREAS OF UNCERTAINTY: The benefit of remote monitoring technologies on patient outcomes has not been established in robust trials. Furthermore, the use of these devices, which can increase the burden of care, has not been integrated into current clinical workflows and electronic medical records. Finally, reimbursement for these telemedicine and remote monitoring services is variable. DATA SOURCES: Literature review. THERAPEUTIC ADVANCES: Advances in digital technology have improved remote monitoring of physiologic parameters relevant to pulmonary medicine. In addition, telemedicine services for the provision of pulmonary rehabilitation and novel hospital-at-home programs have been developed. These new home-based programs have been adapted for COVID-19 and may also be relevant for the management of acute and chronic pulmonary diseases after the pandemic. CONCLUSION: Digital remote monitoring of physiologic parameters relevant to pulmonary medicine and novel hospital-at-home programs are feasible and may improve care for patients with acute and chronic respiratory-related disorders.

Effects of Nintedanib on Quantitative Lung Fibrosis Score in Idiopathic Pulmonary Fibrosis.

BACKGROUND: Nintedanib slows disease progression in patients with Idiopathic Pulmonary Fibrosis (IPF) by reducing decline in Forced Vital Capacity (FVC). The effects of nintedanib on abnormalities on high-resolution computed tomography scans have not been previously studied. OBJECTIVE: We conducted a Phase IIIb trial to assess the effects of nintedanib on changes in Quantitative Lung Fibrosis (QLF) score and other measures of disease progression in patients with IPF. METHODS: 113 patients were randomized 1:1 to receive nintedanib 150 mg bid or placebo double-blind for ≥6 months, followed by open-label nintedanib. The primary endpoint was the relative change from baseline in QLF score (%) at month 6. Analyses were descriptive and exploratory. RESULTS: Adjusted mean relative changes from baseline in QLF score at month 6 were 11.4% in the nintedanib group (n=42) and 14.6% in the placebo group (n=45) (difference 3.2% [95% CI: -9.2, 15.6]). Adjusted mean absolute changes from baseline in QLF score at month 6 were 0.98% and 1.33% in these groups, respectively (difference 0.35% [95% CI: -1.27, 1.96]). Adjusted mean absolute changes from baseline in FVC at month 6 were -14.2 mL and -83.2 mL in the nintedanib (n=54) and placebo (n=54) groups, respectively (difference 69.0 mL [95% CI: -8.7, 146.8]). CONCLUSION: Exploratory data suggest that in patients with IPF, 6 months' treatment with nintedanib was associated with a numerically smaller degree of fibrotic change in the lungs and reduced FVC decline versus placebo. These data support previous findings that nintedanib slows the progression of IPF.

A Case of Pneumonia Caused by Pneumocystis Jirovecii and Cryptococcus Neoformans in a Patient with HTLV-1 Associated Adult T- Cell Leukemia/Lymphoma: Occam's Razor Blunted.

Adult T-cell leukemia/lymphoma (ATLL) is usually preceded by infection with human T-cell lymphotropic virus I (HTLV-I). Patients with ATLL frequently get opportunistic infections of the lungs, intestines, and central nervous system. Pneumocystis pneumonia is commonly known as an AIDS defining illness. Grocott's methenamine silver stain of bronchoalveolar lavage (BAL) samples obtained via bronchoscopy remain the gold standard for diagnosis. Pulmonary cryptococcosis is seen in patients with T-cell deficiencies and a diagnosis is made by culture of sputum, BAL, or occasionally of pleural fluid. We present the second case of coinfection with these two organisms in a patient with ATLL who was successfully treated with trimethoprim-sulfamethoxazole, corticosteroids, and fluconazole. We illustrate the need for high clinical vigilance for seeking out an additional diagnosis, especially in immunocompromised patients if they are not improving despite receiving appropriate treatment.

A preliminary quality of life questionnaire-bronchiectasis: a patient-reported outcome measure for bronchiectasis.

BACKGROUND: The Quality of Life Questionnaire-Bronchiectasis (QOL-B) is the first disease-specific, patient-reported outcome measure for patients with bronchiectasis. Content validity, cognitive testing, responsivity to open-label treatment, and psychometric analyses are presented. METHODS: Reviews of literature, existing measures, and physician input were used to generate the initial QOL-B. Modifications following preliminary cognitive testing (N = 35 patients with bronchiectasis) generated version (V) 1.0. An open-ended patient interview study (N = 28) provided additional information and was content analyzed to derive saturation matrices, which summarized all disease-related topics mentioned by each participant. This resulted in QOL-B V2.0. Psychometric analyses were carried out using results from an open-label phase 2 trial, in which 89 patients were enrolled and treated with aztreonam for inhalation solution. Responsivity to open-label treatment was observed. Additional analyses generated QOL-B V3.0, with 37 items on eight scales: respiratory symptoms; physical, role, emotional, and social functioning; vitality; health perceptions; and treatment burden. For each scale, scores are standardized on a 0-to-100-point scale; higher scores indicate better health-related quality of life. No total score is calculated. A final cognitive testing study (N = 40) resulted in a minor change to one social functioning scale item (QOL-B V3.1). RESULTS: Content validity, cognitive testing, responsivity to open-label treatment, and initial psychometric analyses supported QOL-B items and structure. CONCLUSIONS: This interim QOL-B is a promising tool for evaluating the efficacy of new therapies for patients with bronchiectasis and for measuring symptoms, functioning, and quality of life in these patients on a routine basis. A final psychometric validation study is needed and is forthcoming. TRIAL REGISTRY: ClinicalTrials.gov; No.: NCT00805025; URL: www.clinicaltrials.gov.

Pharmacologic agents for mucus clearance in bronchiectasis.

There are no approved pharmacologic agents to enhance mucus clearance in non-cystic fibrosis (CF) bronchiectasis. Evidence supports the use of hyperosmolar agents in CF, and studies with inhaled mannitol and hypertonic saline are ongoing in bronchiectasis. N-acetylcysteine may act more as an antioxidant than a mucolytic in other lung diseases. Dornase α is beneficial to patients with CF, but is not useful in patients with non-CF bronchiectasis. Mucokinetic agents such as ß-agonists have the potential to improve mucociliary clearance in normals and many disease states, but have not been adequately studied in patients with bronchiectasis.

Pharmacological treatment options for bronchiectasis: focus on antimicrobial and anti-inflammatory agents.

Patients with bronchiectasis experience tenacious mucus, recurrent infectious exacerbations, and progressive worsening of symptoms and obstruction over time. Treatment is aimed at trying to break the cycle of infection and progressive airway destruction. Antibacterial treatment is targeted towards likely organisms or tailored to the results of sputum culture. Inhaled antibacterial therapy may offer the advantage of increased local concentration of medication, while minimizing systemic adverse effects; however, to date, studies have been equivocal in this disorder. Macrolides, in addition to their antibacterial properties, have unique anti-inflammatory properties, which may make them useful in this disorder. Other mucoactive and anti-inflammatory agents, such as inhaled corticosteroids, mannitol and hypertonic saline, may also prove useful in this disease, but further studies are needed.

Bronchiectasis: new findings in the pathogenesis and treatment of this disease.

PURPOSE OF REVIEW: Bronchiectasis is an under-appreciated cause of chronic lung disease in the USA. We highlight developments in diagnosis and treatment of this debilitating disease. RECENT FINDINGS: A possible link between gastroesophageal reflux and development of nontuberculous mycobacterial lung disease was highlighted. Reflux is more common in patients with nontuberculous mycobacterial lung disease, and among those with established bronchiectasis more extensive disease was observed in those patients who also had reflux. Long-term mortality in bronchiectasis was significantly associated with age, lower body mass index, dyspnea, lack of vaccination, hypoxemia, hypercapnia, and other functional parameters. In a large, randomized clinical trial, addition of inhaled tobramycin to ciprofloxacin for acute exacerbations of Pseudomonas infection produced microbiologic improvement correlating with clinical outcomes but not overall improvement. A review noted that five macrolide trials reported reduced sputum volume, improved lung function, and better symptom control. Finally, articles suggested benefit from inhaled hyperosmolar agents (e.g. hypertonic saline and inhaled mannitol). SUMMARY: The possible link between gastroesophageal reflux and nontuberculous mycobacterial lung disease, and the microbiology and resistance patterns of bacteria observed in these patients were clarified. A large study of inhaled tobramycin for exacerbations was inconclusive, but macrolide therapy and hyperosmolar agents hold promise.

Addition of montelukast or salmeterol to fluticasone for protection against asthma attacks: a randomized, double-blind, multicenter study.

BACKGROUND: For patients whose asthma is uncontrolled with low-dose inhaled corticosteroids, addition of alternative therapy instead of increasing the steroid dose is recommended by current treatment guidelines. OBJECTIVE: To compare montelukast, a once-daily leukotriene receptor antagonist, and salmeterol, a twice-daily, long-acting beta-agonist, concomitantly administered with inhaled fluticasone, according to the percentage of patients without an asthma attack for 1 year. METHODS: A randomized, double-blind, double-dummy, multicenter study was conducted. Adult patients with moderate-to-severe persistent asthma (ages 14-73 years) receiving inhaled fluticasone (220 microg/d) who remained symptomatic during a 4-week run-in period were randomized to the addition of salmeterol (84 microg/d) or montelukast (10 mg/d) for 48 weeks. RESULTS: Of the 1,473 randomized patients, 743 were randomized to montelukast and 730 to salmeterol; 1,059 patients completed the study. Eighty percent of patients in the montelukast group and 83.3% of patients in the salmeterol group remained attack free during the 48 weeks of treatment (relative risk, 1.20; 95% confidence interval, 0.96-1.49). Montelukast significantly reduced blood eosinophil counts compared with salmeterol, whereas salmeterol significantly increased prealbuterol forced expiratory volume in 1 second, asthma-specific quality of life, morning peak expiratory flow rate, and decreased nocturnal awakenings compared with montelukast. Differences between treatments were small, and both treatments were generally well tolerated. CONCLUSIONS: Addition of montelukast or salmeterol to an inhaled corticosteroid similarly protected most patients from experiencing an asthma attack during a 1-year period, but, based on noninferiority limits, the study was inconclusive with regard to a difference between treatment groups.

Pulmonary artery sarcoma: a case report of surgical cure and 5-year follow-up.

Pulmonary artery sarcoma is a rare tumor that is frequently misdiagnosed as chronic pulmonary embolism. With heightened clinical awareness and advancement in technology, the diagnosis is now increasingly being made preoperatively. Previous literature has described the disease to be uniformly fatal, with surgical resection as the single most effective modality for short-term palliation. We present the case of a patient in whom pulmonary artery sarcoma was diagnosed preoperatively and who underwent surgical resection with no evidence of recurrence during long-term follow-up, suggesting that early identification and aggressive surgical intervention has the potential to be curative.