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International audience; Lung transplantation (LTx) is the last-resort treatment for end-stage respiratory insufficiency, whatever its origin, and represents a steadily expanding field of endeavor. Major developments have been impelled over the years by painstaking efforts at LTx centers to improve donor and recipient selection, and multifaceted attempts have been made to meet the challenges raised by surgical management, perioperative care, and long-term medical complications. The number of procedures has increased, leading to improved post-LTx prognosis. One consequence of these multiple developments has been a pruning away of contraindications over time, which has, in some ways, complicated the patient selection process. With these considerations in mind, the Francophone Pulmonology Society (Société de Pneumology de Langue Française [SPLF]) has set up a task force to produce up-to-date working guidelines designed to assist pulmonologists in managing end-stage respiratory insufficiency, determining which patients may be eligible for LTx, and appropriately timing LTx-center referral. The task force has examined the most recent literature and evaluated the risk factors that continue to limit patient survival after LTx. Ideally, the objectives of LTx are to prolong life while improving quality of life. The guidelines developed by the task force apply to a limited resource and are consistent with the ethical principles described below.; La transplantation pulmonaire (TxP) constitue le traitement ultime de l’insuffisance respiratoire terminale quelle qu’en soit l’origine et voit son activité en permanente expansion. Le travail minutieux des centres de TxP portant sur la sélection des donneurs et des receveurs d’un greffon pulmonaire ainsi que de l’ensemble des efforts fournis pour relever les défis de la prise en charge chirurgicale, péri-opératoire et la gestion des complications médicales de la TxP à plus long terme ont permis une augmentation du nombre de procédures et l’amélioration du pronostic post-TxP. Les membres du groupe de travail de la Société de pneumologie de langue française (SPLF) ont réalisé une mise à jour des données connues de la littérature et analysé les facteurs de risque qui limitent les chances de survie après la TxP. L’objectif de ce travail a été de guider les pneumologues dans leur prise en charge de l’insuffisance respiratoire terminale, de les aider à identifier les patients potentiellement éligibles à la TxP et de déterminer selon quels délais les adresser à centre de TxP. Les objectifs de la TxP demeurent l’allongement de la vie et l’amélioration de la qualité de vie des malades. Les propositions faites dans ce document portent sur une ressource limitée et restent guidées par des principes éthiques décrits plus loin.

The first COPD biennial organized by the French Society of Respiratory Diseases (SPLF) took place on 17 December 2021.The objective of the biennial was to discuss current knowledge regarding COPD pathophysiology, current treatments, research development, and future therapeutic approaches.The different lecturers laid emphasis on the complexity of pathophysiologic mechanisms including bronchial, bronchiolar and parenchymal alterations, and also dwelt on the role of microbiota composition in COPD pathenogenesis. They pointed out that addition to inhaled treatments, ventilatory support and endoscopic approaches have been increasingly optimized. The development of new therapeutic pathways such as biotherapy and cell therapy (stem cells…) call for further exploration.The dynamism of COPD research was repeatedly underlined, and needs to be further reinforced, the objective being to "understand so as to better innovate" so as to develop effective new strategies for treatment and management of COPD.

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.

Fungal aspergillosis colonization and allergic bronchopulmonary aspergillosis (ABPA) can have a strong impact on the prognosis in cystic fibrosis (CF). We conducted round table discussions involving French experts from pediatric and adult centers caring for patients with CF, microbiologists, radiologists and pharmacists. The aim was to explore the current state of knowledge on: the pathophysiological mechanisms of Aspergillus and other micromycetes infections in CF (such as Scedosporium sp.), and on the clinico-biological diagnosis of ABPA. In perspective, the experts explored the role of imaging in the diagnosis of APBA, specifically CT and MRI; as well as the role of bronchoscopy in the management. We also reviewed the therapeutic management, including different corticosteroid regimens, antifungals and anti-IgE antibodies.The diagnosis of ABPA in CF should be based on more standardized biological assays and imaging to optimize treatment and follow-up.

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.

Introduction La prevention des infections respiratoires basses par la vaccination anti-grippale (AG) et anti-pneumococcique (AP) fait partie de la prise en charge recommandee pour tout patient atteint de BPCO, au meme titre que l’aide au sevrage tabagique et l’activite physique. Elle contribue notamment a limiter le risque d’exacerbations dont l’impact sur les patients, leur evolution et la societe est majeur. Les donnees vaccinales de cohortes francaises sont anciennes. La connaissance des facteurs associes a la vaccination peut contribuer a mieux construire les campagnes d’incitation. L’objectif de ce travail etait d’evaluer le taux de couverture vaccinale AG et AP, et les determinants de la vaccination chez des patients atteints de BPCO pris en charge par les pneumologues. Methodes Les donnees de 3 cohortes ont ete colligees: Colibri et Palomb ont ete lancees en 2012 et 2014 et impliquent des pneumologues hospitaliers et liberaux, Initiatives BPCO a ete lancee en 2004 essentiellement en centres hospitalo-universitaires. Seuls les dossiers renseignes entre 2012 et 2018 ont ete pris en compte par souci d’homogeneite entre les 3 cohortes. La couverture vaccinale rapportee a ete decrite, ses liens avec les donnees cliniques et fonctionnelles respiratoires ont ete analyses de maniere uni- puis multivariee. Resultats Au total 5927 patients (sur 7200) de la multicohorte ainsi constituee avaient des donnees suffisantes pour l’analyse ( Tableau 1 ). L’âge moyen etait de 66 ans, 33 % etaient des femmes, la repartition des stades GOLD 1/2/3/4 etait de 15 %/48 %/28 %/10 %, celle des GOLD A/B/C/D de 31 %/32 %/8 %/21 %, respectivement. Les % de patients avec historique d’asthme, de maladies cardio-vasculaires, de diabete etaient de 9 %, 52 % et 13 %, respectivement. Le tableau rapporte les taux de vaccination AG et AP globalement et par categorie GOLD A/B/C/D determinee a partir des symptomes et de l’historique d’exacerbation. Les analyses multivariees ont montre une association entre les vaccinations AG et AP et l’âge, le statut fumeur (moindre vaccination des fumeurs actifs), la severite de la BPCO (VEMS, exacerbations), les comorbidites (asthme, et pour la vaccination AG HTA et diabete). Conclusion Les taux de vaccination restent tres insuffisants, meme pour les patients suivis en pneumologie. La communication doit porter sur l’importance de ne pas limiter la vaccination a des profils de patients particuliers mais d’en faire beneficier l’integralite des BPCO.

Introduction La sensibilisation aspergillaire et l’aspergillose bronchopulmonaire allergique (ABPA) sont des complications frequentes de la mucoviscidose, et sont associees a une acceleration du declin fonctionnel respiratoire. Peu de donnees existent concernant ces pathologies dans la dilatation de bronches diffuse non mucoviscidosique (DDB) et la dyskinesie ciliaire primitive (DCP). L’objectif de cette etude est de comparer la prevalence de la sensibilisation aspergillaire et de l’ABPA dans une cohorte de patients adultes atteints de DDB, de DCP ou de mucoviscidose. Methodes Une etude transversale monocentrique retrospective a ete realisee chez les patients adultes en etat stable suivis dans le service de pneumologie de l’hopital Cochin (Paris) entre 2014 et 2018. La sensibilisation aspergillaire a ete definie comme une elevation des IgE specifiques aspergillaires (≥ 0,35 kUI/L), sans elevation des IgE totales. L’ABPA a ete definie selon les criteres de la Cystic Fibrosis Foundation [1] . Resultats Au total, 323 patients ont ete inclus : 59 patients atteints de DDB (principalement idiopathiques ou post-infectieuses), 58 patients atteints de DCP, 38 patients mucoviscidosiques suffisants pancreatiques (Muco SP), et 167 patients mucoviscidosiques insuffisants pancreatiques (Muco IP). La prevalence de la sensibilisation aspergillaire etait de 1,7 % dans le groupe DDB diffuses, 10,9 % dans groupe DCP, et 33,6 % dans le groupe mucoviscidose. Les criteres d’ABPA minimale et classique etaient retrouves respectivement chez 1,7 % et 0 % du groupe DDB diffuses, 1,8 % et 0 % du groupe DCP, 5,3 % et 7,9 % du groupe Muco SP et 7,2 % et 11,4 % du groupe Muco IP. Conclusion La prevalence de la sensibilisation aspergillaire et de l’ABPA semble faible chez les adultes atteints de DDB diffuses ou de DCP. Elle est plus frequente chez les patients mucoviscidosiques, en particulier en cas d’insuffisance pancreatique exocrine. La physiopathologie de l’ABPA pourrait etre liee a la dysfonction de la proteine CFTR, caracteristique de la mucoviscidose.

The relations between chronic obstructive pulmonary disease (COPD) and respiratory diseases due to Aspergillus spp. are not well understood.We analysed a retrospective series of patients hospitalized with a diagnosis of COPD and respiratory disease due to Aspergillus. Patients were identified between 2010 and 2015 from the medico-administrative database of Cochin hospital, Paris. Historical, clinical, biological, microbiological and imaging data were collected and described. Diagnoses were reclassified based on reference definitions and classifications from the literature. Patients were classified according to the type of Aspergillus-related diseases and risk factors were described.Forty patients were identified. Classifiable Aspergillus-related respiratory conditions were confirmed in 26 of them including 12 allergic bronchopulmonary aspergillosis (ABPA), 8 chronic pulmonary aspergillosis (CPA), 1 invasive pulmonary aspergillosis (IPA) and 3 diagnostic associations ABPA/CPA. Other respiratory comorbidities were present in all cases of CPA and immunodepression was recorded for semi-invasive and invasive forms. Finally, 16 patients could not be classified, among whom Aspergillus related lung disease was considered as likely in one-half.The complexity of the diagnosis of pulmonary aspergillosis is related to its multiple types with sometimes unclear distinctions. Any type of pulmonary aspergillosis can be observed in patients with COPD, depending on associated risks factors. It would be helpful to establish specific classifications adapted to patients with COPD. This will require larger, prospective, multicentre studies.

Cystic fibrosis (CF) is a genetic disease primarily affecting the lungs, which could lead to chronic respiratory failure and premature death. CF patients are usually followed in specialized centers, but may present outside of these centers when they seek care for acute pulmonary and/or non-pulmonary complications. The aim of this paper is to provide appropriate knowledge necessary for managing respiratory and non-respiratory emergencies in CF adults.The review is based on international guidelines, extensive search of the available literature using Pubmed, and experience of the CF reference center at Cochin hospital (Paris, France). Complications occurring after solid organ transplantation (e.g., lung and/or liver) are excluded from this review.Main acute respiratory complications are pulmonary exacerbations, hemoptysis, pneumothorax and allergic bronchopulmonary aspergillosis. Acute non-respiratory complications include hyponatremic dehydration, acute pancreatitis, acute complications of gallstones, distal intestinal obstruction syndrome, symptomatic nephrolithiasis, acute kidney injury, drug intolerances and catheter-related acute complications.This review summarizes acute pulmonary and non-pulmonary complications occurring in adults with CF, focusing on diagnosis and principles of treatment, with the aim of providing a reference that can be used in clinical practice.

Bronchiectasis is defined by a permanent and irreversible increase in airway caliber. Computed tomography (CT) scan is required for diagnosis and allows differentiating localized (affecting only one lobe) from diffuse (affecting two or more lobes) bronchiectasis. Localized bronchiectasis is usually related to a local cause (e.g., tumor, compression by lymph nodes, foreign body) whereas there are multiple causes of diffuse bronchiectasis. Main causes of diffuse bronchiectasis can be grouped into 5 categories: (1) immune deficiencies; (2) genetic disorders responsible for abnormal mucociliairy clearance (e.g., cystic fibrosis and primary ciliary dyskinesia); (3) autoinflammatory and connective tissue diseases (e.g., rheumatoid arthritis, Sjogren syndrome); (4) chronic airway inflammatory disorders (e.g., allergic broncho-pulmonary aspergillosis) or chronic infections (e.g., by nontuberculous mycobacteria) and; (5) sequelae of pulmonary insults (e.g., infection, radiotherapy). In the absence of a specific cause, bronchiectasis are called idiopathic. Bronchoscopy is the cornerstone for etiological diagnosis in localized bronchiectasis. Investigation of diffuse bronchiectasis includes standardized biological tests, eventually completed by specialized explorations (e.g., analysis of epithelial ion transport and/or ciliary beating). After this systematic approach, an etiological diagnosis is obtained in approximately 60% of cases, often resulting in therapeutic consequences and in some cases in genetic counselling.

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!

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.

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.

Since the discovery of chloride secretion by the Cystic Fibrosis Transport regulator CFTR in 1983, and CFTR gene in 1989, knowledge about CFTR synthesis, maturation, intracellular transfer and function has dramatically expanded. These discoveries have led to the distribution of CF mutations into 6 classes with different pathophysiological mechanisms. In this article we will explore the state of art on CFTR synthesis and its chloride secretion function. We will then explore the consequences of the 6 classes of mutations on CFTR protein function and we will describe the new therapeutic developments aiming at correcting these defects.

Cystic fibrosis (CF) is the most prevalent severe genetic disease in the Caucasian population. It affects approximately 7000 patients in France, of whom 54% are adults (≥18 years old). Improvement in therapeutic management has resulted in a major decrease in pediatric mortality and an increase in the longevity of adults with CF, resulting in a major increase in the number of adult patients over the past 20 years. Demographic forecasts indicate that the French adult CF population will increase by approximately 2000 patients between 2010 and 2025. These data indicate the need for an adaptation of the healthcare system for providing high quality care to all CF patients in the near future.

Introduction Les maladies pulmonaires chroniques post-tabagiques (BPCO et emphyseme) ont une prevalence importante et sont frequemment associees a une hypertension pulmonaire (HTP) legere a moderee. Dans certains cas, ces maladies s’accompagnent d’une HTP severe. L’objectif de notre etude etait de decrire la population de patients atteints d’HTP severe associee aux maladies pulmonaires chroniques post-tabagiques bronchiques ou emphysemateuses. Methodes Nous avons etudie retrospectivement les patients du groupe 3.1 de la classification des HTP (maladies pulmonaires chroniques obstructives), issus de registre national francais des HTP entre 2005 et 2016, et plus particulierement leurs caracteristiques cliniques, fonctionnelles respiratoires, hemodynamiques, leur survie ainsi que l’effet des traitements specifiques de l’HTAP (inhibiteurs de la phosphodiesterase 5 et antagonistes des recepteurs de l’endotheline) sur l’hematose, le catheterisme cardiaque droit et le test de marche de 6 minutes (TM6). Nous avons egalement pratique une analyse tomodensitometrique pour ces patients avec quantification de l’emphyseme et des vaisseaux pulmonaires de petits calibres. Resultats Cinquante-cinq patients ont ete inclus dans l’etude. Il y avait 82 % d’hommes pour un âge moyen de 66 ans. Le VEMS moyen etait de 66 % theorique. Ces patients presentaient une HTP severe (PAPm moyenne = 46 mmHg et IC moyen = 2,31L/min/m2). Les traitements specifiques de l’HTAP entrainaient une amelioration modeste de l’index cardiaque et du TM6, au prix d’une degradation nette de l’hematose, souvent a l’origine d’un arret de traitement. Ces traitements n’etaient pas associes a une meilleure survie. La mediane de survie etait de 40 mois et un VEMS > 80 % theorique etait associe a un mauvais pronostic. Ce sous-groupe de patients avec un VEMS > 80 % theorique presentait une mediane de survie de 20 mois contre 47 mois pour les patients avec VEMS Conclusion Les patients atteints d’HTP severe associee a une maladie pulmonaire chronique post-tabagique ont un pronostic sombre et ne beneficient pas des traitements specifiques de l’HTAP. Il existe un sous-groupe de patients fumeurs avec un trouble ventilatoire obstructif absent ou leger qui presentent une HTP plus severe et un pronostic plus sombre, sans phenotype scanographique particulier decelable.

Introduction Dans un article recent (Eur Resp J 2017 ; 50(5). pii : 1701034), nous avons individualise 5 phenotypes de BPCO et developpe un algorithme d’identification de ces phenotypes par une analyse de type classification and regression tree (CART). Les variables necessaires a cette classification etaient (dans cet ordre) : les comorbidites cardiovasculaires et/ou le diabete, l’echelle de dyspnee MRC, le VEMS ( %), l’âge, et l’indice de masse corporelle. Des differences de survie globale ont ete observees entre les sous-groupes dans plusieurs cohortes de patients, confirmant la pertinence clinique de cette approche. Notre objectif etait de determiner si ces phenotypes sont associes a des differences de capacite a l’exercice lors du test de marche de 6 minutes (TM6). Methodes Cinq cent trente-sept patients de la cohorte initiatives BPCO ayant des donnees completes pour les variables necessaires a la classification (voir-ci-dessus) et un TM6 ont ete analyses. La distance parcourue lors du TM6 a ete analysee comme une variable continue (test de Kruskall–Wallis test) et categorisee (pour une analyse du Chi2) avec un seuil a 350 m (valide pour sa valeur pronostique). Resultats La distance parcourue lors du TM6, analysee en variable continue ou en deux classes, etait significativement differente entre les phenotypes (chaque analyse, p Fig. 1 ). Conclusion Les phenotypes de patients atteint de BPCO identifies par notre algorithme sont associes a des differences de capacite a l’exercice, confortant la pertinence clinique de ces phenotypes.