[Natural history of COPD: evolution of concepts]. / Histoire naturelle de la BPCO : évolution des concepts.

NATURAL HISTORY OF COPD: EVOLUTION OF CONCEPTS. Chronic obstructive pulmonary disease (COPD) is a common chronic respiratory disease responsible for significant morbidity and mortality. The natural history of this disease is complex. The first cause is tobacco smoke exposure, followed by exposure to biomass smoke and occupational exposure to inhaled toxic substances. Genetic predisposing factors are known as the alpha-1-antitrypsin deficiency. The involvement of respiratory events in childhood in the genesis of the disease is also increasingly described. The decline of the different respiratory trajectories can be accelerated in COPD. The phenotypes of this disease also have a role in the evolution of respiratory function over time and their descriptions can modulate the therapeutic management in this disease.

HISTOIRE NATURELLE DE LA BPCO: ÉVOLUTION DES CONCEPTS. La bronchopneumopathie chronique obstructive (BPCO) est une maladie respiratoire chronique fréquente et responsable d'une morbi-mortalité importante. La compréhension de l'histoire naturelle de cette pathologie a évolué ces dernières années. La première cause de BPCO est le tabagisme, suivi de l'exposition à la fumée de biomasse et de l'exposition professionnelle à des toxiques inhalés. Des facteurs de prédisposition génétique sont connus, comme le déficit en alpha-1-antitrypsine. L'implication d'événements respiratoires au cours de l'enfance dans la genèse de la maladie est également de plus en plus décrite. Le déclin des différentes trajectoires de la fonction respiratoire peut être accéléré en cas de BPCO. Les phénotypes de cette maladie jouent aussi un rôle dans l'évolution de la fonction respiratoire au cours du temps et leur description peut moduler la prise en charge thérapeutique de cette maladie.

[COPD exacerbations]. / Exacerbations de BPCO.

COPD EXACERBATIONS. COPD exacerbations, characterized by an acute worsening of dyspnea and/or cough and sputum volume beyond daily variations, are a public health issue because of their frequency, the number of hospitalizations and the high rate of recurrence. Because of non-specific symptoms, differential diagnoses must be ruled out. Additional tests are rarely necessary, and treatment is outpatient, except in severe cases (respiratory distress, severity of COPD, socio-economic conditions). Increased bronchodilator treatment, and sometimes antibiotic and/or systemic corticosteroid therapy, are the main measures to be introduced and should be systematically reassessed. After an exacerbation, the aim is to optimize follow-up and treatment, to prevent recurrences.

EXACERBATIONS DE BPCO. Les exacerbations de bronchopneumopathie chronique obstructive (EBPCO), caractérisées par une aggravation aiguë de la dyspnée et/ou de la toux et du volume de l'expectoration au-delà des variations quotidiennes, constituent un enjeu de santé publique du fait de leur fréquence, du nombre d'hospitalisations et du taux élevé de récidives. L'absence de spécificité des symptômes impose d'éliminer les diagnostics différentiels. Les examens complémentaires sont rarement nécessaires, et le traitement est ambulatoire hormis en cas de signes de gravité (détresse respiratoire, sévérité de la BPCO, conditions socio-économiques). La majoration du traitement bronchodilatateur, parfois une antibiothérapie et/ou une corticothérapie systémique sont les principales mesures à instaurer et doivent faire l'objet d'une réévaluation systématique. Après une exacerbation, l'optimisation du suivi et du traitement a pour objectif la prévention d'une récidive.

Case report: Extracorporeal life support as a successful bridge to recovery in an incident case of pulmonary arterial hypertension.

Pulmonary arterial hypertension (PAH) is characterized by a progressive increase in pulmonary vascular resistance (PVR) due to vascular remodeling of the small pulmonary arteries. In advanced RV failure or severe hypoxemia, extra corporeal life support (ECLS) is now to be considered, with the objective to bridge patients back to their baseline clinical state while waiting or right after lung transplantation, or bridge to pharmacological optimization of PAH (i.e., bridge to recovery). We describe herein a case of a 30-year-old woman (gravida 6, para 6) with an incident case of heritable PAH revealed by refractory hypoxemia. Despite the use of mechanical ventilation and fluid optimization, the patient remained profoundly hypoxemic. ECLS was then initiated to avoid tissue hypoxia. The mechanical option chosen was peripheral femoro-femoral venoarterial extracorporeal membrane oxygen (VA-ECMO), percutaneously implanted. Due to the absence of evidence of chronic respiratory disease or chronic thromboembolic pulmonary hypertension, this severe pre-capillary pulmonary hypertension was attributed to PAH. Therefore, epoprostenol infusion and an association of oral treatments (bosentan and tadalafil) were administered. A dramatic improvement was observed, allowing decannulation 7 days after the initiation of pharmacological treatment. After 29 days, the patient was discharged from the hospital with epoprostenol, bosentan, and tadalafil. The assessment has been completed by positive research on mutations (c.741C > G, p.Tyr247) corresponding to a loss of function of the bone morphogenetic protein receptor 2 (BMPR2) gene. The final diagnosis was heritable PAH. The use of ECLS has been well demonstrated in patients with PAH complicated by acute RV failure or refractory hypoxemia in the "bridge-to-transplantation" strategy. Only a few reports have described the use of ECLS as a "bridge-to-recovery" with PAH drugs in untreated or undertreated PAH patients, but none has described such a rapid improvement with resolution of refractory hypoxemia. More studies are needed to assess the benefits and limitations of the "bridge-to-recovery" strategy and to identify the patients most likely to benefit from it.