Although idiopathic pulmonary fibrosis remains a devastating diagnosis, recent advances have improved our understanding of many facets of this disease. These breakthroughs, combined with the increased general availability of therapeutic trials, hold the promise of a brighter future for idiopathic pulmonary fibrosis patients. For example, we now have a more comprehensive understanding of the diagnostic criteria and natural history of the disease. Several studies have shown that simple measurement of pulmonary physiology or gas exchange can be used to predict patient survival. By identifying several molecular pathways that play significant roles in the pathogenesis of idiopathic pulmonary fibrosis, investigators have produced a growing list of novel potential therapeutic targets for the disease. Several prospective, controlled therapeutic trials have been conducted. Others are ongoing or are still in the planning stages. These efforts have advanced our current knowledge of idiopathic pulmonary fibrosis and have raised new important questions, as well as having generated the interest and momentum needed to gain additional ground in the fight against this challenging disease. This article offers the reader a view of the recent advances in idiopathic pulmonary fibrosis research, with a focus on natural history, pathogenesis and treatment.

Keywords: Pulmonary fibrosis/diagnosis; Pulmonary fibrosis/drug therapy; Fibroblasts; Transforming growth factor beta; Lung/pathology, Anti-inflammatory agents/therapeutic use

In patients with myositis, the lung is commonly involved, and the presence of anti-aminoacyl-tRNA synthetase (anti-ARS) antibodies marks the presence or predicts the development of interstitial lung disease (ILD). A distinct clinical entity-antisynthetase syndrome-is characterized by the presence of anti-ARS antibodies, myositis, ILD, fever, arthritis, Raynaud's phenomenon, and mechanic's hands. The most common anti-ARS antibody is anti-Jo-1. More recently described anti-ARS antibodies might confer a phenotype that is distinct from that of anti-Jo-1-positive patients and is characterized by a lower incidence of myositis and a higher incidence of ILD. Among patients with antisynthetase syndrome-related ILD, the response to immunosuppressive medications is generally, but not universally, favorable.

Keywords: Lung diseases, interstitial; Pneumonia; Infection.

Objective: Lymphangioleiomyomatosis (LAM) is a rare, destructive disease of the lungs with a limited number of determinants of disease activity, which are a critical need for clinical trials. FGF23 has been implicated in several chronic pulmonary diseases. We aimed to determine the association between serum FGF23 levels and pulmonary function in a cohort of patients with LAM. Methods: This was a descriptive single-center study in which subjects with LAM and controls with unreported lung disease were recruited. Serum FGF23 levels were measured in all subjects. Clinical data, including pulmonary function testing, were retrospectively obtained from electronic medical records of LAM subjects. Associations between FGF23 levels and clinical features of LAM were explored via nonparametric hypothesis testing. Results: The sample comprised 37 subjects with LAM and 16 controls. FGF23 levels were higher in the LAM group than in the control group. In the LAM group, FGF23 levels above the optimal cutoff point distinguished 33% of the subjects who had nondiagnostic VEGF-D levels. Lower FGF23 levels were associated with impaired DLCO (p = 0.04), particularly for those with isolated diffusion impairment with no other spirometric abnormalities (p = 0.04). Conclusions: Our results suggest that FGF23 is associated with pulmonary diffusion abnormalities in LAM patients and elicit novel mechanisms of LAM pathogenesis. FGF23 alone or in combination with other molecules needs to be validated as a biomarker of LAM activity in future clinical research.

Keywords: Lymphangioleiomyomatosis; Fibroblast growth factor-23; Pulmonary diffusing capacity.

The diagnosis and correction of a rare case of traumatic transpericardial diaphragmatic hernia is presented with spontaneous intraoperative reduction by the verification of the lesions of the left lateral wall of the pericardium, diaphragmatic pericardium, and the central tendon, with intact left hemidiaphragm. Only one such case has been published in the last thirty years.

Keywords: Transpericardial diaphragmatic hernia. Thoracic injuries

Lung volume recruitment involves deep inflation techniques to achieve maximum insufflation capacity in patients with respiratory muscle weakness, in order to increase peak cough flow, thus helping to maintain airway patency and improve ventilation. One of these techniques is air stacking, in which a manual resuscitator is used in order to inflate the lungs. Although intrathoracic pressures can rise considerably, there have been no reports of respiratory complications due to air stacking. However, reaching maximum insufflation capacity is not recommended in patients with known structural abnormalities of the lungs or chronic obstructive airway disease. We report the case of a 72-year-old woman who had poliomyelitis as a child, developed torsion scoliosis and post-polio syndrome, and had periodic but infrequent asthma attacks. After performing air stacking for 3 years, the patient suddenly developed a pneumothorax, indicating that this technique should be used with caution or not at all in patients with a known pulmonary pathology.

Keywords: Barotrauma; Pneumothorax; Insufflation.

Sleep studies conducted at an altitude that is different from the home altitude can yield misleading results regarding the severity of obstructive sleep apnea (OSA). The objective of the present study was to determine the sleep characteristics of a patient undergoing polysomnography (PSG) in three Colombian cities at different altitudes (Bogotá, at 2,640 m above sea level [ASL]; Bucaramanga, at 959 m ASL; and Santa Marta, at 15 m ASL). The patient was an obese man with diabetes and suspected OSA. All PSG recordings were scored and interpreted in accordance with American Academy of Sleep Medicine criteria. In Bogotá, PSG revealed moderate OSA (an apnea-hypopnea index [AHI] of 21 events/h); in Bucaramanga, PSG revealed increased upper airway resistance (an AHI of 2 events/h); in Santa Marta, PSG revealed mild OSA (an AHI of 7 events/h). The reduction in the AHI was predominantly a reduction in hypopneas and obstructive apneas. The respiratory events were shorter in duration in the city at an intermediate altitude. Given that the AHI varied widely across cities, we can assume that the patient is normal or has moderate OSA depending on the city where he is. Central apneas were found to have no influence on the AHI.

Keywords: Sleep apnea, obstructive; Polysomnography; Altitude.

Objective: To determine the prevalence of asthma in a group of obese adult candidates for bariatric surgery and to evaluate the severity of asthma in this group of patients. Methods: This was a cross-sectional study involving 363 obese adults (body mass index ≥ 35 kg/m2) evaluated by a pulmonologist, using clinical evaluation as a diagnostic tool for asthma. All patients underwent clinical evaluation and spirometry and were divided into two groups (asthma and control). The patients with asthma were stratified by the severity of asthma. Results: The prevalence of asthma in the obese population studied was 18.5% (95% CI: 14.5-22.4). That prevalence was 20.4% (95% CI: 16.2-24.5) and 13.7% (95% CI: 10.1-17.2) in the women and the men, respectively. Asthma symptoms in the last twelve months were present in 8.0% (95% CI: 5.2-10.7), and the initial manifestation of asthma symptoms occurred during childhood/adolescence in 17.4% (95% CI: 13.5-21.3). In the asthma group, intermittent asthma was present in 29 patients (43.3%), mild persistent asthma in 7 (10.4%), moderate asthma in 25 patients (37.3%), and severe persistent asthma in 6 (9.0%). Conclusions: Using clinical evaluation as the diagnostic criterion, we found the prevalence of asthma to be high in this group of obese adults. Asthma was more common in females, and the initial manifestation of asthma symptoms more commonly occurred during childhood/adolescence. The severity of asthma in this group of obese adults was within the range of mean values predicted for the general population. Intermittent asthma, mild persistent asthma, and moderate persistent asthma predominated.

Keywords: Asthma; Cross-sectional studies; Spirometry; Respiratory function tests; Obesity, morbid.

Objective: Impaired respiratory mechanics and gas exchange may contribute to sleep disturbance in patients with COPD. We aimed to assess putative associations of different domains of lung function (airflow limitation, lung volumes, and gas exchange efficiency) with polysomnography (PSG)-derived parameters of sleep quality and architecture in COPD. Methods: We retrospectively assessed data from COPD 181 patients = 40 years of age who underwent spirometry, plethysmography, and overnight PSG. Univariate and multivariate linear regression models predicted sleep efficiency (total sleep time/total recording time) and other PSG-derived parameters that reflect sleep quality. Results: The severity of COPD was widely distributed in the sample (post-bronchodilator FEV1 ranging from 25% to 128% of predicted): mild COPD (40.3%), moderate COPD (43.1%), and severe-very severe COPD (16.6%). PSG unveiled a high proportion of obstructive sleep apnea (64.1%) and significant nocturnal desaturation (mean pulse oximetry nadir = 82.2% ± 6.9%). After controlling for age, sex, BMI, apnea-hypopnea index, nocturnal desaturation, comorbidities, and psychotropic drug prescription, FEV1/FVC was associated with sleep efficiency (ß = 25.366; R2 = 14%; p < 0.001), whereas DLCO predicted sleep onset latency (ß = -0.314; R2 = 13%; p < 0.001) and rapid eye movement sleep time/total sleep time in % (ß = 0.085; R2 = 15%; p = 0.001). Conclusions: Pulmonary function variables reflecting severity of airflow and gas exchange impairment, adjusted for some potential confounders, were weakly related to PSG outcomes in COPD patients. The direct contribution of the pathophysiological hallmarks of COPD to objectively measured parameters of sleep quality seems to be less important than it was previously assumed.

Keywords: Pulmonary disease, chronic obstructive; Respiratory function tests; Sleep; Sleep apnea, obstructive; Comorbidity.

Objective: To assess prescription patterns for short-acting ß2 agonists (SABAs) and other asthma medications in asthma patients treated by specialists and participating in the SABA use IN Asthma (SABINA) study in Brazil. Methods: This was an observational, cross-sectional study conducted at five sites in different regions of Brazil. The primary endpoints were to record SABA prescriptions and obtain data on over-the-counter (OTC) SABA purchases at the pharmacy. Results: Data on 218 asthma patients were analyzed. Of those 218 patients, 80.3% were prescribed SABAs in addition to their maintenance therapy, with a mean of 11.2 SABA canisters in the previous 12 months. Of those patients, 71.4% were prescribed = 3 canisters and 42.2% were prescribed = 10 canisters. None of the patients were prescribed SABA monotherapy. A total of 14.2% of the patients reported purchasing SABAs OTC at a pharmacy without a prescription. Of those, 48.4% purchased = 3 SABA canisters. A fixed-dose combination of an inhaled corticosteroid and a long-acting ß2 agonist was prescribed to 95.0% of the patients. In the year before the study visit, 45.0% of the patients received at least one course of oral corticosteroid burst treatment. Asthma was well controlled in 43.1% of the patients, partly controlled in 34.9%, and uncontrolled in 22.0%. Patients reported a mean of 1.1 severe asthma exacerbations, with 49.1% experiencing 1 or more severe exacerbations. Conclusions: Overprescription and OTC purchases of SABAs are common in Brazil, possibly leading to the need for courses of oral corticosteroids. The health care community should collaborate to implement evidence-based recommendations and promote health education to improve asthma management in Brazil.

Keywords: Asthma; Brazil; Bronchodilator agents; Prescriptions