Defining the "Frequent Exacerbator" Phenotype in COPD: A Hypothesis-Free Approach.

Background: The COPD "frequent exacerbator" phenotype is usually defined by at least two treated exacerbations per year and is associated with a huge impact on patient health. However, existence of this phenotype and corresponding thresholds still need to be formally confirmed by statistical methods analyzing exacerbation profiles with no specific a priori hypothesis. The aim of this study was to confirm the existence of the frequent exacerbator phenotype with an innovative unbiased statistical analysis of prospectively recorded exacerbations.

Mast cells are associated with exacerbations and eosinophilia in children with severe asthma.

The role of mast cells in the pathogenesis of childhood asthma is poorly understood. We aimed to estimate the implication of airway mucosal mast cells in severe asthma and their relationship with clinical, functional, inflammatory and remodelling parameters.Bronchial biopsies were performed in 36 children (5-18 years) with severe asthma: 24 had frequent severe exacerbations and/or daily symptoms in the previous year (symptomatic group), and 12 had few symptoms and a persistent obstructive pattern (paucisymptomatic group).

[Features of asthma in women: what is the relationship with hormonal status?].

Introduction: The prevalence and control of asthma are modulated by hormonal changes in women, suggesting an influence of sex hormones on the airways.

Background: The blood levels of both oestrogens and progesterone can modulate airway tone and inflammation. Asthma prevalence changes at puberty and the menopause, events also associated with modifications of adipose tissue and behaviour.

[Lung is also involved in juvenile dermatomyositis].

Juvenile dermatomyositis is the leading cause of chronic idiopathic inflammatory myopathy of auto-immune origin in children. Lung involvement in inflammatory myopathies is well described in adults, involving mostly interstitial lung disease, aspiration pneumonia and alveolar hypoventilation. We propose to describe its specificities in children.

Clinical characteristics and quality of life in women with COPD: an observational study.

Background: The impact of COPD on patient's quality of life is well established, but gender differences have received little attention.

Methods: To describe factors associated with the health-related quality of life by gender: A cross-sectional observational study (NCT01007734) was conducted in COPD patients followed by pulmonologists. The first patient included had to be a woman.

Oxidative stress-mediated iNKT-cell activation is involved in COPD pathogenesis.

Chronic obstructive pulmonary disease (COPD) is a major clinical challenge mostly due to cigarette smoke (CS) exposure. Invariant natural killer T (iNKT) cells are potent immunoregulatory cells that have a crucial role in inflammation. In the current study, we investigate the role of iNKT cells in COPD pathogenesis.

Crohn's disease and nontropical endomyocardial fibrosis.

The pathogenesis of endomyocardial fibrosis (EMF) is poorly understood. EMF may result from autoimmune scarring of the endocardium. Clinically, EMF presents as a restrictive cardiomyopathy.

Small airways diseases, excluding asthma and COPD: an overview.

This review is the summary of a workshop on small airways disease, which took place in Porquerolles, France in November 2011. The purpose of this workshop was to review the evidence on small airways (bronchiolar) involvement under various pathophysiological circumstances, excluding asthma and chronic obstructive pulmonary disease. Histopathological patterns associated with small airways disease were reviewed, including cellular and obliterative bronchiolitis.

[Mild asthma in children: new data and a revival of interest].

According to the Global Initiative for Asthma (GINA) classification, mild asthma includes intermittent and mild persistent asthma. It represents more than 75% of asthmatic children. The symptoms and functional impact are well described.

The lung is involved in juvenile dermatomyositis.

Background: Juvenile dermatomyositis (JDM) is the main cause of chronic idiopathic inflammatory myopathy of autoimmune origin in children. The aim of this multicenter prospective study was to describe respiratory status and treatment of children followed for JDM.

Methods And Patients: Clinical manifestations, pulmonary function tests (PFT), chest high-resolution computed tomography (HRCT) scan results, and treatments and their adverse effects were analyzed in children followed for JDM.

[The distal airways in systemic disease].

The association of inflammatory involvement of the distal airways or bronchiolitis and systemic diseases is essentially observed in Sjögren's syndrome, rheumatoid arthritis and chronic inflammatory bowel disease. Bronchiolitis may be mainly cellular in nature, often involving lympho-monocytic cells, and sometimes associated with lymphoid follicles, as in Sjögren's syndrome. It may also, particularly in rheumatoid arthritis, be constrictive, with peribronchiolar fibrosis.

Difficult childhood asthma: management and future.

Diagnosis and management of severe asthma implies the definition of different entities, that is, difficult asthma and refractory severe asthma, but also the different phenotypes included in the term refractory severe asthma. A complete evaluation by a physician expert in asthma is necessary, adapted for each child. Identification of mechanisms involved in different phenotypes in refractory severe asthma may improve the therapeutic approach.

Do we need exercise tests to detect gas exchange impairment in fibrotic idiopathic interstitial pneumonias?

In patients with fibrotic idiopathic interstitial pneumonia (f-IIP), the diffusing capacity for carbon monoxide (DLCO) has been used to predict abnormal gas exchange in the lung. However, abnormal values for arterial blood gases during exercise are likely to be the most sensitive manifestations of lung disease. The aim of this study was to compare DLCO, resting PaO(2), P(A-a)O(2) at cardiopulmonary exercise testing peak, and oxygen desaturation during a 6-min walk test (6MWT).

[Asthma: treatment of exacerbations].

Introduction: Exacerbations remain, in both adults and children, a common reason for emergency consultation. The management of the asthmatic patient with an acute exacerbation is well defined.

Background: The initial evaluation, based on the background risk factors and the clinical examination, will determine the choice of treatment and management.

Frequent and widespread vascular abnormalities in human signal transducer and activator of transcription 3 deficiency.

Background: Signal transducer and activator of transcription 3 (STAT3) deficiency is responsible for autosomal dominant hyperimmunoglobulin E syndrome, characterized by recurrent bacterial and fungal infections, connective tissue abnormalities, hyperimmunoglobulin E, and Th17 lymphopenia. Although vascular abnormalities have been reported in some patients, the prevalence, characteristics, and etiology of these features have yet to be described.

Methods And Results: We prospectively screened 21 adult STAT3-deficient patients [corrected] (median age, 26 years; range, 17-44 years) [corrected] for vascular abnormalities.

[When to consider COPD and how to assess it?].

Detected in 30% of COPD patients and underdiagnosed, COPD must be systematically investigated in all about 40 years-old smokers, without waiting for clinical symptoms, late and inconstant. The diagnosis is made with spirometry. Search for a status of "frequent exacerbations", co-morbidities (including cardio-vascular disease), evaluation of dyspnea intensity with a scale (MRC), assessment of disease impact on daily life, allow to identify risk groups and to target the therapeutic management overall.

[Exacerbations of asthma in pregnant women].

Prevention of exacerbations and effective treatment is essential in pregnant asthmatic women. The management is well documented. Misunderstanding of the recommendations leads to unsuitable, insufficient treatment and is responsible for more frequent recurrences in the pregnant woman compared with the non pregnant.

[Structural abnormalities and inflammation in COPD: a focus on small airways].

Chronic obstructive pulmonary disease (COPD) is characterized by poorly reversible airflow limitation associated with airway remodelling and inflammation of both large and small airways. The site of airflow obstruction in COPD is located in the small airways, justifying a focus on this compartment. The structural abnormalities that are found in bronchioles with a diameter less than 2mm are characterized by increased airway wall thickness with peribronchial fibrosis, and by luminal obstruction by mucous exudates.