Inspiratory muscle training (IMT) has been described as one of the components of the treatment of chronic lung conditions such as obstructive and restrictive lung diseases. Although the number of studies showing results of IMT in patients with interstitial lung disease (ILD) is scarce when compared with studies in patients with COPD, evidence points to benefits of IMT in this population. This scoping review aimed to explore the role and the rationale of IMT in patients with ILD and to gather recent evidence on the effects of IMT in this population. The studies included in this review showed improvements in respiratory muscle function, quality of life, exercise capacity and dyspnea after ILD patients participated in programs that included stand-alone IMT or combined with pulmonary rehabilitation. There is still a gap in the literature to allow a clear conclusion on the indications of IMT as part of ILD treatment because of poor research design and small numbers of participants. Therefore, although IMT seems to have a positive effect in patients with ILD, current evidence prevents us from drawing a definite conclusion. Further studies need to be conducted using better research methodology to demonstrate and confirm the positive effects of IMT.

Keywords: Respiratory muscles; Lung diseases, interstitial; Rehabilitation.

Objective: To determine whether inspiratory muscle training can increase strength and endurance of these muscles in quadriplegic patients. Methods: Eight quadriplegic patients (7 males and 1 female) with injury to the lower cervical spine (segments C4-C7) were submitted to inspiratory muscle training with a threshold inspiratory muscle trainer adjusted to 30% of MIP. The training sessions were carried out with the patients in a sitting position, 5 days a week for 8 weeks. Endurance time, MIP, MEP and FVC were determined at baseline, week 4 and week 8. Results: In comparison with the mean baseline value, there was an increase in MIP, measured in the sitting position, at weeks 4 and 8 (−83.0 ± 18.9 cmH2O vs. −104.0 ± 19.4 cmH2O and −111.3 ± 22.7 cmH2O). There was an increase in MEP, also in the sitting position, at week 4 (from 36.8 ± 8.1 to 42.6 ± 8.8 cmH2O). There was an improvement in FVC at week 4 (from 2.1 ± 0.8 to 2.5 ± 0.6 L, representing an increase of 24 ± 22%). Although there was an increase in endurance (sitting) at week 8, the difference was not significant in comparison with the baseline value (29.8 ± 21.0 vs. 35.9 ± 15.5 min, an increase of 173 ± 233%). Conclusions: Quadriplegic patients can benefit from training at low loads (30% of MIP), which can improve inspiratory muscle strength, FVC and expiratory muscle performance.

Keywords: Respiratory mechanics; Quadriplegia; Breathing exercises.

Objective: The aim of the present study was to evaluate the effects that inspiratory muscle training (IMT) and respiratory exercises have on muscle strength, peak expiratory flow (PEF) and severity variables in children with asthma. Methods: This was a randomized analytical study involving 50 children with asthma allocated to one of two groups: an IMT group, comprising 25 children submitted to IMT via an asthma education and treatment program; and a control group, comprising 25 children who were submitted only to monthly medical visits and education on asthma. The IMT was performed using a pressure threshold load of 40% of maximal inspiratory pressure (MIP). The results were evaluated using analysis of variance, the chi-square test and Fisher's exact test, values of p > 0.05 being considered significant. Results: In the comparative analysis, pre- and post-intervention values of MIP, maximal expiratory pressure (MEP) and PEF increased significantly in the IMT group: MIP from −48.32 ± 5.706 to −109.92 ± 18.041 (p < 0.0001); MEP from 50.64 ± 6.55 to 82.04 ± 17.006 (p < 0.0001); and PEF from 173.6 ± 50.817 to 312 ± 54.848 (p < 0.0001). In the control group, however, there were no significant differences between the two time points in terms of MIP or MEP, although PEF increased from 188 ± 43.97 to 208.80 ± 44.283 (p < 0.0001). There was a significant improvement in the severity variables in the IMT group (p < 0.0001). Conclusions: Programs involving IMT and respiratory exercises can increase mechanical efficiency of the respiratory muscles, as well as improving PEF and severity variables.

Keywords: Breathing exercises; Asthma/therapy; Asthma/rehabilitation.

Objective: Respiratory muscle weakness is a functional repercussion of chronic lung disease (CLD). The objective of this study was to assess the effects of home-based respiratory muscle training (RMT) in children and adolescents with CLD or neuromuscular disease (NMD). Methods: This was a quasi-experimental study involving children and adolescents with CLD or NMD. Before and after 6 months of home-based RMT, we measured respiratory muscle strength (MIP and MEP), PEF, and peak cough flow (PCF). We made statistical comparisons between the pre-RMT and post-RMT values, as well as evaluating the correlation between the duration and effect of RMT. Results: The study included 29 patients, with a mean age of 12 years (range, 5-17 years), of whom 18 (62.1%) were male. The CLD group comprised 11 patients (37.9%), and the NMD group comprised 18 (62.1%). The mean duration of the RMT was 60 weeks (range, 46-90 weeks) in the CLD group and 39 weeks (range, 24-89 weeks) in the NMD group. In comparison with the pre-RMT values, the post-RMT values for MIP and MEP were significantly higher in both groups, whereas those for PEF and PCF were significantly higher only in the NMD group. We found no correlation between the duration and the effect of RMT. Conclusions: Home-based RMT appears to be an effective strategy for increasing respiratory muscle strength in children and adolescents with CLD or NMD, although it increased the ability to cough effectively only in those with NMD.

Keywords: Breathing exercises; Cystic fibrosis; Bronchiolitis obliterans; Neuromuscular diseases; Cough.

Objective: To investigate the efficacy of upper limb exercise training (ULExT) in improving the performance of activities of daily living (ADL) that involve the upper limbs (UL) in patients with COPD. Methods: The Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines were used in this systematic review. PubMed and EBSCOhost databases were searched to identify randomized controlled trials involving adults with COPD who underwent ULExT, compared with those who underwent other types of exercise or no exercise, in order to assess the performance of ADL that involve the UL. The methodological quality of the selected studies was assessed using the Physiotherapy Evidence Database scale. Results: Five studies, with a total sample of 173 subjects, met the inclusion criteria. The results of the selected studies showed that ULExT is safe and can significantly improve the performance of ADL that involve the UL in patients with COPD. However, there were inconsistencies in the results, especially regarding the perception of symptoms during ADL. The small number of studies included and their methodological quality do not allow for firm conclusions. Conclusions: The findings of this review revealed that ULExT is a safe therapeutic approach and can improve the performance of ADL that involve the UL in patients with COPD, but the results are unclear. Further investigation through well-designed randomized trials is warranted to determine the effectiveness of ULExT in improving the performance of ADL that involve the UL in patients with COPD.

Keywords: Upper extremity; Pulmonary disease, chronic obstructive; Activities of daily living, Exercise therapy.

The objectives of this study were to characterize and clarify the relationships between the various cognitive domains affected in COPD patients and the disease itself, as well as to determine the prevalence of impairment in the various cognitive domains in such patients. To that end, we performed a systematic review using the following databases: PubMed, Scopus, and ScienceDirect. We included articles that provided information on cognitive impairment in COPD patients. The review of the findings of the articles showed a significant relationship between COPD and cognitive impairment. The most widely studied cognitive domains are memory and attention. Verbal memory and learning constitute the second most commonly impaired cognitive domain in patients with COPD. The prevalence of impairment in visuospatial memory and intermediate visual memory is 26.9% and 19.2%, respectively. We found that cognitive impairment is associated with the profile of COPD severity and its comorbidities. The articles reviewed demonstrated that there is considerable impairment of the cognitive domains memory and attention in patients with COPD. Future studies should address impairments in different cognitive domains according to the disease stage in patients with COPD.

Keywords: Pulmonary disease, chronic obstructive; Mild cognitive impairment; Hypoxia, brain.

Respiratory bronchiolitis-associated interstitial lung disease is one of many within the spectrum of smoking-related diffuse infiltrative lung diseases. The clinical and functional characteristics are typically subtle. Herein, we describe two cases of diagnosed through open-lung biopsy, and characterized by insidious evolution of dyspnea, digital clubbing, cystic lesions on computed tomography scans, and hipoxemia upon exertion. We emphasize that, when smokers are evaluated, it is imprtant to consider a diagnosis of respiratory bronchiolitis-associated interstitial lung disease in the context of interstitial cystitis, as well as in that of lymphangioleiomyomatosis, eosinophilic granuloma and idiophatic pulmonary fibrosis.

Keywords: Smoking. Interstitial lung diseases. Bronchiolitis.

Many interstitial lung diseases (ILDs) share mechanisms that result in a progressive fibrosing phenotype. In Brazil, the most common progressive fibrosing interstitial lung diseases (PF-ILDs) are chronic hypersensitivity pneumonitis, idiopathic pulmonary fibrosis, unclassified ILD, and connective tissue diseases. PF-ILD is seen in approximately 30% of patients with ILD. Because PF-ILD is characterized by disease progression after initiation of appropriate treatment, a diagnosis of the disease resulting in fibrosis is critical. Different criteria have been proposed to define progressive disease, including worsening respiratory symptoms, lung function decline, and radiological evidence of disease progression. Although the time elapsed between diagnosis and progression varies, progression can occur at any time after diagnosis. Several factors indicate an increased risk of progression and death. In the last few years, antifibrotic drugs used in patients with idiopathic pulmonary fibrosis have been tested in patients with PF-ILD. The effects of nintedanib and placebo have been compared in patients with PF-ILD, a mean difference of 107.0 mL/year being observed, favoring nintedanib. The U.S. Food and Drug Administration and the Brazilian Health Regulatory Agency have approved the use of nintedanib in such patients on the basis of this finding. Pirfenidone has been evaluated in patients with unclassified ILD and in patients with other ILDs, the results being similar to those for nintedanib. More studies are needed in order to identify markers of increased risk of progression in patients with ILD and determine the likelihood of response to treatment with standard or new drugs.

Keywords: Alveolitis, extrinsic allergic; Idiopathic pulmonary fibrosis; Lung diseases, interstitial; Connective tissue diseases.

Connective tissue disease-associated interstitial lung disease (CTD-ILD) represents a group of systemic autoimmune disorders characterized by immune-mediated organ dysfunction. Systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myositis, and Sjögren's syndrome are the most common CTDs that present with pulmonary involvement, as well as with interstitial pneumonia with autoimmune features. The frequency of CTD-ILD varies according to the type of CTD, but the overall incidence is 15%, causing an important impact on morbidity and mortality. The decision of which CTD patient should be investigated for ILD is unclear for many CTDs. Besides that, the clinical spectrum can range from asymptomatic findings on imaging to respiratory failure and death. A significant proportion of patients will present with a more severe and progressive disease, and, for those, immunosuppression with corticosteroids and cytotoxic medications are the mainstay of pharmacological treatment. In this review, we summarized the approach to diagnosis and treatment of CTD-ILD, highlighting recent advances in therapeutics for the various forms of CTD.

Keywords: Lung diseases, interstitial; Collagen diseases; Scleroderma, systemic; Arthritis, rheumatoid; Myositis; Therapeutics.

Objective: A substantial number of people with COPD suffer from exacerbations, which are defined as an acute worsening of respiratory symptoms. To minimize exacerbations, telehealth has emerged as an alternative to improve clinical management, access to health care, and support for self-management. Our objective was to map the evidence of telehealth/telemedicine for the monitoring of adult COPD patients after hospitalization due to an exacerbation. Methods: Bibliographic search was carried in PubMed, Cumulative Index to Nursing and Allied Health Literature (CINAHL), Web of Science, Scopus, Biblioteca Virtual de Saúde/LILACS and Cochrane Library databases to identify articles describing telehealth and telemonitoring strategies in Portuguese, English, or Spanish published by December of 2021. Results: Thirty-nine articles, using the following concepts (number of articles), were included in this review: telehealth (21); telemonitoring (20); telemedicine (17); teleconsultation (5); teleassistance (4); telehomecare and telerehabilitation (3 each); telecommunication and mobile health (2 each); and e-health management, e-coach, telehome, telehealth care and televideo consultation (1 each). All these concepts describe strategies which use telephone and/or video calls for coaching, data monitoring, and health education leading to self-management or self-care, focusing on providing remote integrated home care with or without telemetry devices. Conclusions: This review demonstrated that telehealth/telemedicine in combination with telemonitoring can be an interesting strategy to benefit COPD patients after discharge from hospitalization for an exacerbation, by improving their quality of life and reducing re-hospitalizations, admissions to emergency services, hospital length of stay, and health care costs.

Keywords: Pulmonary disease, chronic obstructive; Symptom flare up; Telemedicine; Patient discharge.

Objective: Fungal pulmonary infections are a significant complication in lung cancer, adversely affecting prognosis and treatment outcomes. This meta-analysis aimed to estimate the prevalence of chronic pulmonary aspergillosis (CPA) and Pneumocystis jirovecii pneumonia (PJP) in lung cancer patients and to identify associated clinical predictors. Methods: A systematic search of EBSCOhost, Embase, PubMed/MEDLINE, Scopus, and Web of Science retrieved 2,823 records, of which 7 studies were eligible (PROSPERO: CRD42024551104). Meta-analyses of proportions and dichotomous and continuous variables were performed using R (meta package) via Jamovi and RevMan 5, with statistical significance set at p<0.05. Results: Among 15,901 lung cancer patients, 177 had CPA and 135 had PJP. The pooled prevalence was 1% for CPA and 23% for PJP. CPA was significantly associated with male sex, smoking, COPD, interstitial lung disease, tuberculosis, and squamous cell carcinoma, and negatively associated with adenocarcinoma. CPA patients also had significantly lower BMI. Bilobectomy, radiotherapy, and concurrent chemoradiotherapy were additional risk factors for CPA. High-dose corticosteroid use (=20 mg/day) was significantly associated with PJP. Conclusion: CPA occurs in a clinically distinct subset of lung cancer patients with identifiable risk factors, while PJP appears to be strongly linked to immunosuppressive therapy. Improved screening strategies are warranted to mitigate the burden of these infections in vulnerable lung cancer populations.

Keywords: lung cancer; fungal infection; pulmonary infection; aspergillosis; Pneumocystis jirovecii; pneumonia.

The initial evaluation of patients with interstitial lung disease (ILD) primarily involves a comprehensive, active search for the cause. Autoantibody assays, which can suggest the presence of a rheumatic disease, are routinely performed at various referral centers. When interstitial lung involvement is the condition that allows the definitive diagnosis of connective tissue disease and the classical criteria are met, there is little debate. However, there is still debate regarding the significance, relevance, specificity, and pathophysiological role of autoimmunity in patients with predominant pulmonary involvement and only mild or benign symptoms of connective tissue disease. The purpose of this article was to review the current knowledge of autoantibody positivity and to discuss its possible interpretations in patients with ILD and without clear etiologic associations, as well as to enhance the understanding of the natural history of an allegedly new disease and to describe the possible prognostic implications. We also discuss the proposition of a new term to be used in the classification of ILDs: lung-dominant connective tissue disease.

Keywords: Idiopathic interstitial pneumonias; Autoantibodies; Connective tissue diseases; Autoimmune diseases; Diagnosis, differential.

Objective: To investigate parameters of lung function and respiratory muscle strength in different stages of Parkinson's disease (PD), as well as to determine their correlation with motor function and quality of life. Methods: This was a cross-sectional study conducted at a referral center for PD in the city of Recife, Brazil. Respiratory muscle strength and lung function, as well as their relationship with motor function and quality of life, were evaluated in patients with PD, stratified by the level of severity, and were compared with the data obtained for a control group. After confirming the normality of data distribution, we performed one-way ANOVA with a post hoc t-test. Results: The sample comprised 66 individuals, in two groups: PD (n = 49) and control (n = 17). All of the parameters investigated showed inverse correlations with PD severity, and there were significant differences among the levels of severity, as well as between the PD and control groups, in terms of the MIP, MEP, FVC, FEV1, and FEF25-75%. The lung function parameters also showed moderate to weak inverse correlations with bradykinesia and rigidity. On a quality of life questionnaire, the total score and mobility domain score both presented a moderate inverse correlation with FVC, FEV1, PEF, and MEP. Conclusions: Respiratory muscle strength and some lung function parameters are impaired from the early stages of PD onward, bradykinesia and rigidity being the cardinal signs that correlate most strongly with impairment of those parameters. Such alterations negatively affect the quality of life of patients with PD.

Keywords: Parkinson disease; Respiratory mechanics; Maximum respiratory pressures; Quality of life.

Drug-induced lung disease (DILD) encompasses a broad, highly heterogeneous group of conditions that may occur as a result of exposure to numerous agents, such as antineoplastic drugs, conventional or biological disease-modifying antirheumatic drugs, antiarrhythmics, and antibiotics. Between 3% and 5% of prevalent cases of interstitial lung diseases are reported as DILDs. The pathogenesis of lung injury in DILD is variable, multifactorial, and often unknown. Acute presentation is the most common, can occur from days to months after the start of treatment, and ranges from asymptomatic to acute respiratory failure. The CT patterns are varied and include ground-glass opacities, organizing pneumonia, and diffuse alveolar damage. Notably, there are no clinical manifestations or CT patterns specific to DILD, which makes the diagnosis quite challenging and necessitates a high index of suspicion, as well as the exclusion of alternative causes such as infection, cardiac-related pulmonary edema, exacerbation of a preexisting ILD, and neoplastic lung involvement. Discontinuation of the offending medication constitutes the cornerstone of treatment, and corticosteroid treatment is usually necessary after the onset of clinical manifestations. The prognosis varies widely, with high mortality rates in severe cases. A history of medications related to pulmonary toxicity in patients with new-onset respiratory symptoms should prompt consideration of DILD as a potential underlying cause.

Keywords: Lung diseases, interstitial/chemically induced; Lung diseases, interstitial/diagnostic imaging; Immunotherapy/adverse effects.