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Resume Introduction La relation entre la bronchopneumopathie obstructive (BPCO) et les differents types d’affections aspergillaires reste mal connue. Materiel et methodes Il s’agit d’une serie retrospective de cas de patients hospitalises entre 2010 et 2015 avec un diagnostic de BPCO et d’affection aspergillaire, identifies a partir de la base de donnees PMSI (programme de medicalisation des systemes d’information) de l’hopital Cochin, Paris. Les donnees historiques, cliniques, biologiques, microbiologiques et d’imagerie ont ete analysees de maniere descriptive. Les diagnostics ont ete reajustes a partir des definitions et classifications de reference de la litterature. Les patients ont ete classes selon le type d’affection aspergillaire et les facteurs de risques associes ont ete decrits. Resultats Quarante patients ont ete inclus. Un diagnostic d’affection aspergillaire a ete retenu chez 24 d’entre eux et reparti en 12 aspergilloses broncho-pulmonaires allergiques (ABPA), 8 aspergilloses pulmonaires chroniques (APC), 1 aspergillose pulmonaire invasive (API) et 3 associations diagnostiques ABPA/APC. Les facteurs de risques associes etaient les antecedents respiratoires pour l’APC et l’immunodepression pour les formes semi-invasives et invasives. Aucun facteur associe a l’ABPA n’etait isole. Un total de 16 patients restaient inclassables avec un diagnostic d’affection aspergillaire probable chez la moitie d’entre eux. Conclusion La complexite du diagnostic d’affection aspergillaire pulmonaire est liee a la multiplicite de ses formes aux frontieres parfois minces. Tous les types d’affection aspergillaire peuvent etre observes chez le patient atteint de BPCO selon les facteurs de risque associes. Des criteres diagnostiques specifiquement adaptes a cette population sont souhaitables. Leur developpement necessitera des etudes plus larges, multicentriques et prospectives.

Cystic fibrosis (CF) is a genetic disease with pulmonary involvement being predominant and often leading to respiratory failure and premature death. Non-pulmonary complications related to CF transmembrane conductance regulator (CFTR) defect are numerous and account for significant morbidity (exocrine pancreatic insufficiency, diabetes, CF-related liver disease, chronic sinusitis, osteoporosis). Improvement in patients' care led to a continuous increase in life expectancy, with a subsequent increase in the number of adult CF patients worldwide. Increased life expectancy comes with increased prevalence of CF-related comorbidities, but also with new emerging complications directly related to ageing (chronic kidney disease, cardiovascular risk factors, cancers). CFTR modulators might also contribute to modify the face of CF epidemiology and prognosis. Ageing with CF has become a challenge for CF patients and caregivers. This review summarizes classic and emerging comorbidities in the context of current growth and ageing of the CF population. It also addresses potential roles of CFTR modulators.

The concept of personalised medicine is recent but the underlying notions are not new: knowing how to adapt care to patients' characteristics is one of the components of the "art of medicine". The advances of science allow to refine considerably the applications of the concept in many fields of medicine including COPD: research has identified phenotypes, endotypes and treatable traits. Personalisation can be applied to all components of care. For instance, the decision to perform screening spirometry relies not only on risk factors (age, smoking, other exposures) but also on symptoms. Assessment of comorbidities often associated with COPD is based on risk factors and their combinations, variable between individuals. Rehabilitation and its components are in essence highly individualised, which a major condition for their success. Last but not least, personalisation of pharmacological therapy, which has long been rather poor, could not benefit from biomarkers of interest (predictive of response), such as blood eosinophil count. Practical strategies using these still need to be established, and new biomarkers may usefully enrich the collection!

Resume Introduction La mucoviscidose est une maladie genetique affectant principalement les voies respiratoires et pouvant conduire a une insuffisance respiratoire chronique et au deces precoce. Si les patients mucoviscidosiques sont habituellement suivis dans des centres specialises, la survenue de complications aigues respiratoires et/ou non respiratoires peut necessiter une prise en charge urgente en dehors de ces centres. L’objectif de cet article est de fournir au medecin non specialiste de la mucoviscidose les elements necessaires a la prise en charge des urgences respiratoires et non respiratoires chez les adultes mucoviscidosiques. Methodes Le contenu de la revue a ete redige a partir des recommandations internationales, des donnees issues d’une recherche documentaire approfondie de la litterature realisee a l’aide de Pubmed, et de l’experience du centre de reference de la mucoviscidose de l’hopital Cochin (Paris). Les complications survenant au decours d’une transplantation d’organe solide (poumon et/ou foie), ne sont pas abordees. Resultats Les principales complications aigues respiratoires de la mucoviscidose sont l’exacerbation respiratoire, l’hemoptysie, le pneumothorax, et l’aspergillose bronchopulmonaire allergique. Les complications aigues non respiratoires incluent la deshydratation aigue hyponatremique, la pancreatite aigue, la pathologie lithiasique biliaire, le syndrome d’obstruction intestinale distale, les lithiases renales, l’insuffisance renale aigue, les intolerances medicamenteuses et les complications associees aux catheters intraveineux. Conclusion Les elements essentiels du diagnostic de ces complications et les principes generaux de leur prise en charge sont detailles avec l’objectif de fournir une reference utilisable en pratique.

Background Breathlessness is the prominent symptom of chronic obstructive pulmonary disease (COPD). Despite optimal therapeutic management including pharmacological and non-pharmacological interventions, many COPD patients exhibit significant breathlessness. Chronic breathlessness is defined as breathlessness that persists despite optimal treatment of the underlying disease. Because of the major disability related to chronic breathlessness, symptomatic treatments including opioids have been recommended by several authors. The prevalence of chronic breathlessness in COPD and its management in routine clinical practice have been poorly investigated. Our aim was to examine prevalence, associated characteristics and management of chronic breathlessness in patients with COPD recruited in a real-life tertiary hospital-based cohort. Methods A prospective study was conducted among 120 consecutive COPD patients recruited, in stable condition, at Nancy University Hospital, France. In parallel, 88 pulmonologists of the same geographical region were asked to respond to an on-line questionnaire on breathlessness management. Results Sixty four (53%) patients had severe breathlessness (modified Medical Research Council scale≥3), despite optimal inhaled medications for 94% of them; 40% had undergone pulmonary rehabilitation within the past 2 years. The severity of breathlessness increased with increasing airflow limitation. Breathlessness was associated with increased symptoms of anxiety, depression and with osteoporosis. No relation was found with other symptoms, exacerbation rate, or cardiovascular comorbidities. Among the patients with chronic breathlessness and Hospitalized Anxiety and/or Depression score > 10, only 25% were treated with antidepressant or anxiolytic. Among the pulmonologists 46 (52%) answered to the questionnaire and expressed a high willingness to prescribe opioids forchronic breathlessness, which contrasted with the finding that none of these patients received such treatments against breathlessness. Conclusion Treatment approaches to breathlessness and associated psychological distress are insufficient in COPD. This study highlights underuse of pulmonary rehabilitation and symptomatic treatment for breathlessness. Electronic supplementary material The online version of this article (10.1186/s12890-019-0851-5) contains supplementary material, which is available to authorized users.

International audience; Chronic obstructive pulmonary disease (COPD) is the chronic respiratory disease with the most important burden on public health in terms of morbidity, mortality and health costs. For patients, COPD is a major source of disability because of dyspnea, restriction in daily activities, exacerbation, risk of chronic respiratory failure and extra-respiratory systemic organ disorders. The previous French Language Respiratory Society (SPLF) guidelines on COPD exacerbations were published in 2003. Using the GRADE methodology, the present document reviews the current knowledge on COPD exacerbation through 4 specific outlines: (1) epidemiology, (2) clinical evaluation, (3) therapeutic management and (4) prevention. Specific aspects of outpatients and inpatients care are discussed, especially regarding assessment of exacerbation severity and pharmacological approach.

The natural history of cystic fibrosis (CF) may be associated both with acute respiratory complications (respiratory exacerbations, haemoptysis, pneumothorax) and with non-respiratory complications (distal intestinal obstruction syndrome, dehydration) that may result in hospitalizations. The aim of this article is to describe the main therapeutic approaches that are adopted in the management of acute complications occurring in CF adults, and to discuss indications for admission of these patients to intensive care units. Adult CF patients admitted to intensive care unit often benefit from antibiotic courses adapted to their chronic bronchial infection, especially when the hospitalization is related to respiratory disease (including haemoptysis and pneumothorax). Nutritional support, including hypercaloric diet, control of hyperglycemia and pancreatic enzyme supplementation is warranted. The recommended therapy for major haemoptysis is bronchial artery embolization. Patient with significant pneumothorax should have a chest tube inserted, while the treatment of distal intestinal obstruction syndrome will most often be medical. In case of respiratory failure, non-invasive ventilation is the preferred mode of ventilatory support because invasive ventilation is associated with poor outcomes. Therapeutic options should always have been discussed between the patient, family members and the CF medical team to allow for informed decision making.

Resume Introduction L’objectif de cet article est de passer en revue les donnees disponibles concernant les liens existant entre les phenotypes des patients presentant une bronchopneumopathie chronique obstructive (BPCO) et leur profil inflammatoire. Etat des connaissances L’inflammation bronchique proximale est associee a la bronchite chronique, l’inflammation et le remodelage des petites voies aeriennes au niveau d’obstruction. La tomodensitometrie permet un phenotypage des patients selon la predominance bronchique ou emphysemateuse des anomalies morphologiques. Les exacerbations, favorisees par une inflammation basale persistante elevee, sont associees au pronostic et a une augmentation de l’inflammation. Des etudes au long cours documentant une relation entre marqueurs inflammatoires (ou effet des anti-inflammatoires) et distension dynamique pourraient confirmer le role promoteur de l’inflammation sur cette distension. Le nombre de cellules inflammatoires presentes dans la paroi des arteres pulmonaires est correle a l’importance de la dysfonction endotheliale pulmonaire. Le risque de developper une hypertension pulmonaire semble augmenter avec l’inflammation systemique de bas grade. Le role de cette inflammation dans les comorbidites de la BPCO (atteinte nutritionnelle et musculaire) est debattu. L’exercice physique regulier pourrait permettre de la reduire. Conclusions Une meilleure connaissance des relations entre phenotype clinique et inflammation pourrait favoriser l’optimisation des traitements actuels et a venir.

Introduction La mucoviscidose est habituellement diagnostiquee dans les premieres annees de vie. Observations Nous rapportons trois cas familiaux de mucoviscidose diagnostiques a l’âge adulte. Le premier patient, âge de 39 ans, a consulte pour une infertilite par atresie des canaux deferents ; le diagnostic de mucoviscidose a ete retenu devant la positivite du test de la sueur et la presence de deux mutations du gene CFTR. La mere de ce patient presentait des surinfections bronchiques a repetition depuis l’enfance. Le test de la sueur etait normal, la mesure de la difference de potentiel nasal transepithelial etait evocatrice de la maladie et la recherche des 33 mutations les plus frequentes de CFTR ne retrouvait qu’une seule mutation. Le sequencage complet du gene CFTR qui a montre une seconde mutation, et affirme le diagnostic de mucoviscidose a 74 ans. L’enquete familiale a revele un cas de mucoviscidose pauci-symptomatique chez un frere du cas index. Conclusions Ces observations illustrent les difficultes du diagnostic des formes attenuees de mucoviscidose a l’âge adulte. Nous discutons les modalites du diagnostic ainsi que l’interet de ce diagnostic pour adapter les therapeutiques et pour le conseil genetique.

L’histoire naturelle de la BPCO est caracterisee par un declin rapide du VEMS et une mortalite precoce. L’un des objectifs des therapeutiques de la BPCO est de modifier l’histoire naturelle de la maladie. La definition de criteres fiables et reproductibles permettant l’evaluation de l’effet des therapeutiques sur l’histoire naturelle de la maladie est necessaire.

La BPCO a longtemps ete consideree comme une maladie respiratoire isolee. Les etudes recentes indiquent cependant que les patients atteints de BPCO ont souvent d’autres pathologies chroniques (ou comorbidites). Ces comorbidites ont un impact important sur les symptomes, le pronostic vital et les couts de traitement. Une prise en charge therapeutique integrant les differentes pathologies presentes chez un meme patient pourrait ameliorer l’histoire naturelle de la BPCO.

Background We have previously modelled future trends in CF demography by calculating flows of patients entering and exiting CF cohorts (Burgel et al. Eur Resp J 2015). These forecasts, which were based on the assumption that major demographic trends remained stable over time, suggested a major increase in the number of CF adults in western European countries by 2025. Objectives To examine the effects of large variations in CF mortality or incidence on estimated numbers of CF patients. Methods Using the flow method on data from the French CF Registry from 2003 to 2012, we estimated numbers of CF children and adults by 2025 (main analysis). Because this analysis assumed that flows remained stable, we examined the impact large variations in CF incidence or mortality on future numbers of CF patients. Results In 2012, there were 6145 CF patients (3099 children; 3046 adults) in the French CF Registry. Applying average flows to these data, the number of CF patients will increase to 8344 patients (3534 children; 4810 adults) by 2025. Assuming a 50% decrease in pediatric and adult mortality, the number of CF patients in 2025 will increase to 8911 patients (+567 patients; +7%) including 3601 children (+67 children; +2%) and 5310 adults (+500 adults; +10%) as compared with the main analysis. Assuming a decrease in CF incidence by 30%, the estimated number of CF patients in 2025 will be 7969 patients (–375 patients; –4%) including 3159 children (–442 children; –12%) and 4810 adults (unchanged). Conclusion Large variations in CF mortality or incidence would have only minimal impact on the major increase in the number of CF adults that is expected in France by 2025.