Different Faces of Idiopathic Pulmonary Fibrosis With Preserved Forced Vital Capacity.

Introduction: Idiopathic pulmonary fibrosis (IPF) is progressive and irreversible. Some discrepancies about IPF staging exists, especially in mild phases. Forced vital capacity (FVC) higher than 80% has been considered early or mild IPF even for the design of clinical trials.

Mapping IPF helps identify geographic regions at higher risk for disease development and potential triggers.

Background And Objective: The relationship between IPF development and environmental factors has not been completely elucidated. Analysing geographic regions of idiopathic pulmonary fibrosis (IPF) cases could help identify those areas with higher aggregation and investigate potential triggers. We hypothesize that cross-analysing location of IPF cases and areas of consistently high air pollution concentration could lead to recognition of environmental risk factors for IPF development.

Detection of Autoantibodies in Bronchoalveolar Lavage in Patients with Diffuse Interstitial Lung Disease.

Introduction: Serum autoantibodies support the diagnosis of interstitial lung disease (ILD) related to systemic autoimmune diseases (SAD-ILD). Nevertheless, their presence in the bronchoalveolar lavage (BAL) has not been explored.

Objectives: To demonstrate the presence of autoantibodies in the BAL of ILD patients at onset of clinical evaluation, its relation with serum autoantibodies and to analyze clinical features of patients with autoantibodies in BAL.

Lung Cancer in Patients With Combined Pulmonary Fibrosis and Emphysema and Idiopathic Pulmonary Fibrosis. A Descriptive Study in a Spanish Series.

Introduction: Information on the association of lung cancer (LC) and combined pulmonary fibrosis and emphysema (CPFE) is limited and derived almost exclusively from series in Asian populations. The main objective of the study was to assess the impact of LC on survival in CPFE patients and in patients with idiopathic pulmonary fibrosis (IPF).

Methods: A retrospective study was performed with data from patients with CFPE and IPF diagnosed in our hospital over a period of 5 years.

Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias.

Pleuroparenchymal fibroelastosis (PPFE) is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs). PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT), chemotherapy, and recurrent respiratory infections.

Pulmonary hemodynamic profile in chronic obstructive pulmonary disease.

Introduction: Few data are available in regards to the prevalence of pulmonary hypertension (PH) in the broad spectrum of COPD. This study was aimed at assessing the prevalence of PH in a cohort of COPD patients across the severity of airflow limitation, and reporting the hemodynamic characteristics at rest and during exercise.

Methods: We performed a retrospective analysis on COPD patients who underwent right-heart catheterization in our center with measurements obtained at rest (n=139) and during exercise (n=85).

Pleuroparenchymal fibroelastosis: is it also an idiopathic entity?

Pleuroparenchymal fibroelastosis (PPFE) is a rare disease that has been recently included in the updated consensus on idiopathic interstitial pneumonias. It shares some clinical features with other chronic interstitial pneumonias (dyspnea, dry cough), and is radiologically characterized by pleural and subpleural parenchymal fibrosis and elastosis, mainly in the upper lobes. The main histological findings include pleural fibrosis and prominent subpleural and parenchymal fibroelastosis.

Chronic obstructive pulmonary disease and left ventricle.

Several studies have shown that the interaction between chronic obstructive pulmonary disease (COPD) and cardiovascular comorbidity is complex and bidirectional, since each of these diseases complicates the prognosis of the other. Recent advances in imaging technology have led to better characterization of cardiac chambers and allowed the relationship between certain cardiac function parameters and COPD clinical and functional variables to be explored. Although cardiac abnormalities in COPD have been mainly associated with the right ventricle, several studies have reported that the left ventricle may also be affected in this disease.

[Adult pulmonary Langerhans' cell histiocytosis: approach to the reality of the Spanish population].

Background And Objective: Pulmonary Langerhans cell histiocytosis (PLCH) is a rare respiratory disease closely associated with smoking. The aim of the study is to know the reality of PLCH in the Catalonian population.

Material And Methods: We included all patients diagnosed of PLCH in 8 different Catalonian hospitals from April 2011 to December 2012.

Echocardiographic abnormalities in patients with COPD at their first hospital admission.

Cardiovascular disease accounts for significant morbidity and mortality in chronic obstructive pulmonary disease (COPD). Its prevalence and mechanisms of association have not been elucidated. The study aimed to assess the prevalence of echocardiographic abnormalities and potential risk factors in patients with COPD at their first exacerbation requiring hospital admission.

Combined Pulmonary Fibrosis and Emphysema Syndrome: A New Phenotype within the Spectrum of Smoking-Related Interstitial Lung Disease.

Combined pulmonary fibrosis and emphysema (CPFE) is a recently defined syndrome, in which centrilobular and/or paraseptal emphysemas in upper lung zones coexist with pulmonary fibrosis in lower lobes in individuals. These patients have a characteristic lung function profile, with unexpected subnormal dynamic and static lung volumes, contrasting with a significant reduction of carbon monoxide transfer (DL(co)) and exercise hypoxemia. Pulmonary hypertension is highly prevalent in CPFE and is the leading determinant of death.

Anemia is a mortality predictor in hospitalized patients for COPD exacerbation.

Background: Anemia is a recognized prognostic factor in many chronic illnesses, but there is limited information about its impact on outcomes in patients hospitalized for acute COPD exacerbation (AECOPD).

Aim: To investigate whether anemia exerts an effect on mortality in patients admitted for AECOPD after one year of follow-up. Methods.

Nutritional status and eating disorders: neglected risks factor for nontuberculous mycobacterial lung disease?

Nontuberculous mycobacterial lung disease (NTMLD) in immunocompetent patients is an increasingly important epidemiologic concern. However, risk factors associated with susceptibility to NTMLD are not completely known. A prevalence of NTMLD appears to be rising, mainly in some populations such as middle-aged or elderly thin women, (a group including those with Lady Windermere syndrome) with neither remarkable history of respiratory disease nor smoking habit.

[Study of the BMPR2 gene in patients with pulmonary arterial hypertension].

Introduction: Mutations of the gene that code bone morphogenic protein type 2 receptor (BMPR2) are involved in the pathogenesis of pulmonary arterial hypertension (PAH), both in its familial (FPAH) and its idiopathic (IPAH) forms.

Method: With the aim of increasing the knowledge of these genetic factors in our area, the BMPR2 gene was studied in 17 patients with PAH, 8 with FPAH and 9 with sporadic IPAH. Additionally, a study was made to see whether the presence of BMPR2 mutations was associated with changes in the CO diffusing CO (DL(CO)) with the aim of evaluating the interest in this measurement in the pre-clinical diagnosis.