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Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated., Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale., Results: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis., Conclusion: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

While asthma patients' treatment adherence (TA) generally leaves to be desired, few data exist on TA evolution from age group to another. During the meeting of a working group of pneumo-pediatricians and adult pulmonologists, we reviewed the literature on adherence according to age group, examined explanations for poor adherence, and explored ways of improving adherence via new technologies. Asthma is a chronic disease for which TA is particularly low, especially during adolescence, but also among adults. Inhaled medications are the least effectively taken. Several explanations have been put forward: cost and complexity of treatments, difficulties using inhalation devices, poor understanding of their benefits, erroneous beliefs and underestimation of the severity of a fluctuating disease, fear of side effects, neglect, and denial (especially among teenagers). Poor TA is associated with risks of needless treatment escalation, aggravated asthma with frequent exacerbations, increased school absenteeism, degraded quality of life, and excessive mortality. Better compliance is based on satisfactory relationships between caregivers and asthmatics, improved caregiver training, and more efficient transmission to patients of relevant information. The recent evolution of innovative digital technologies opens the way for enhanced communication, via networks and dedicated applications, and thanks to connected inhalation devices, forgetfulness can be limited. Clinical research will also help to ameliorate TA. Lastly, it bears mentioning that analysis of the existing literature is hampered by differences in terms of working definitions and means of TA measurement., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Background: Since the previous French guidelines were published in 2017, substantial additional knowledge about idiopathic pulmonary fibrosis has accumulated., Methods: Under the auspices of the French-speaking Learned Society of Pulmonology and at the initiative of the coordinating reference center, practical guidelines for treatment of rare pulmonary diseases have been established. They were elaborated by groups of writers, reviewers and coordinators with the help of the OrphaLung network, as well as pulmonologists with varying practice modalities, radiologists, pathologists, a general practitioner, a head nurse, and a patients' association. The method was developed according to rules entitled "Good clinical practice" in the overall framework of the "Guidelines for clinical practice" of the official French health authority (HAS), taking into account the results of an online vote using a Likert scale., Results: After analysis of the literature, 54 recommendations were formulated, improved, and validated by the working groups. The recommendations covered a wide-ranging aspects of the disease and its treatment: epidemiology, diagnostic modalities, quality criteria and interpretation of chest CT, indication and modalities of lung biopsy, etiologic workup, approach to familial disease entailing indications and modalities of genetic testing, evaluation of possible functional impairments and prognosis, indications for and use of antifibrotic therapy, lung transplantation, symptom management, comorbidities and complications, treatment of chronic respiratory failure, diagnosis and management of acute exacerbations of fibrosis., Conclusion: These evidence-based guidelines are aimed at guiding the diagnosis and the management in clinical practice of idiopathic pulmonary fibrosis., (Copyright © 2022 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: The aim of the study was to assess, compliance with maintenance treatment in patients with persistent asthma in clinical practice., Methods: This observational, cross-sectional, prospective, multicenter study was conducted in metropolitan France on patients with persistent asthma under the care of a representative sample of general practitioners and pneumologists. Compliance was determined via access to the Health Insurance database allowing comparison between the actual consumption of medications and the corresponding theoretical consumption related to prescription. Additional compliance data were obtained using the validated Morisky questionnaire., Results: Eighty one physicians included 488 patients, mean age of 51 years, 55% women. The statement of consumption (SC) was available for 35% of patients however the Morisky questionnaire (present for 85% of patients) showed a similar compliance between patients with or without SC. The good compliance rate (compliance ≥80%) was observed in 51% based on the SC and in 38% based on the Morisky questionnaire. Among possible predictive factors, only obesity was associated with a bad compliance (19% vs 7%, P<0.05). Patients with poor compliances displayed more severe symptoms and a higher rate of non-control (21.7% vs 5.7%, P<0.01)., Conclusions: The statement of consumption is a useful tool to provide quantitative measurement of compliance in daily practice., (Copyright © 2018 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

The key pathophysiological feature of chronic obstructive pulmonary disease (COPD) is an abnormal inflammatory bronchial reaction after inhalation of toxic substances. The priority is the avoidance of such toxic inhalations, but the use of anti-inflammatory drugs also seems appropriate, especially corticosteroids that are the sole anti-inflammatory drug available for this purpose in France. The risks associated with the prolonged use of these parenteral drugs are well known. Inhalation is therefore the optimal route, but inhaled drugs may also lead to adverse consequences. In COPD, there is an inhaled corticosteroids overuse, and a non-satisfactory respect of the guidelines. Consequently, their withdrawal should be considered. We reviewed seven clinical studies dealing with inhaled corticosteroids withdrawal in patients with COPD and found that included populations were heterogenous with different concomitant treatments. In non-frequent exacerbators receiving inhaled corticosteroids outside the recommendations, withdrawal appears to be safe under a well-managed bronchodilator treatment. In patients with severe COPD and frequent exacerbations, the risk of acute respiratory event is low when they receive concomitant optimal inhaled bronchodilators. However, other risks may be observed (declining lung function, quality of life) and a discussion of each case should be performed, especially in case of COPD and asthma overlap., (Copyright © 2017 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Clinical trials have provided some evidence of a favorable effect of inhaled corticosteroids on the frequency of exacerbations and on the quality of life of patients with chronic obstructive pulmonary disease (COPD). In contrast, ICS have little or no impact on lung function decline and on mortality., State of the Art: Inhaled corticosteroids are recommended only in a minority of COPD patients, those with severe disease and repeated exacerbations and probably those with the COPD and asthma overlap syndrome. However, surveys indicate that these drugs are inappropriately prescribed in a large population of patients with COPD. Overtreatment with inhaled corticosteroids exposes these patients to an increased risk of potentially severe side-effects such as pneumonia, osteoporosis, and oropharyngeal candidiasis. Moreover, it represents a major waste of health-care spending., Conclusion: Primary care physicians as well as pulmonologists should be better aware of the benefits as well as the side-effects and costs of inhaled corticosteroids., (Copyright © 2016 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Objectives: To estimate the prevalence of treated chronic obstructive pulmonary disease (COPD) and its associated costs by stage of severity., Methods: The study was conducted on the 2011 data of the french general beneficiary sample database (EGB). EGB is a 1/97th sample of the whole population of the beneficiaries of the main compulsory national health insurances. COPD cases and the level of severity of the disease have been identified using new algorithms established from the available parameters in EGB. Costs were estimated using a collective perspective., Results: The minimal prevalence of treated COPD was estimated at 3.8% in patients of 40 years and older and 1.9% regardless of the age of individuals. This population was predominantly male (58.2%) with a mean age of 68.8 years (±12.7). A total of 6.2% of patients had a health-care utilization suggestive of a very severe stage of COPD and 8.1%, 13.8% and 71.9% suggestive of severe, moderate and mild stages respectively. Over one year, 28.8% of patients visited a specialist respiratory physician, 5.0% were hospitalized (≥24h) for COPD and 6.7% died. Patients experienced an average of 1.7 (±1.5) exacerbations per year and only 61.4% received specific pharmacological treatment for COPD during the year. The average yearly health-care cost of a patient with COPD was estimated at €9382, with €5342 directly related to COPD., Conclusion: This study based on medico-administrative databases confirms the high epidemiological and economic burden of COPD in France., (Copyright © 2014 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Alpha-1 antitrypsin, secreted by the liver, inhibits neutrophil elastase. Its deficiency favours the development of emphysema. Restoring a "protective" serum level in deficient patients should make it possible to inhibit the development of emphysema., State of the Art: Human plasma-derived alpha-1 antitrypsin is a blood-derived drug sold in France under the name Alfalastin(®). The recommended posology is an I.V. administration of 60 mg/kg once a week. Human plasma-derived alpha-1 antitrypsin restores anti-elastase protection in the lower lung and prevents experimental emphysema induced by the elastasis of human neutrophils in hamster. The low number of patients with alpha-1 antitrypsin deficiency is one of the difficulties to perform sufficiently powerful randomised studies. However, randomised studies have reported the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on mortality, FEV1 decline and the frequency of exacerbations. Randomised control trials have demonstrated the efficacy of human plasma-derived alpha-1 antitrypsin perfusions on the loss of lung density assessed by CT scan., Conclusion: Augmentation therapy is simple in its conception and implementation, but it is expensive. However, there are currently no other solutions., (Copyright © 2014 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Chronic obstructive pulmonary disease (COPD) is underdiagnosed because of limited disease awareness with trivialization of the symptoms in the general population., Methods: A survey was conducted in a representative sample (n=2758) of individuals older than 40 years of age in the general population of France. Respiratory symptoms and knowledge about COPD were assessed in individuals with or at risk for COPD (n=860, 31% of the sample)., Results: In the overall sample, 40% of individuals had a Medical Research Council dyspnea grade of 1 or more but only 9% spontaneously reported shortness of breath. Of these 9%, 72% reported limitations to their daily activities but only 14% believed they had severe lung disease. In the overall sample, only 220 (8%) individuals knew the term COPD and only 66% associated the term COPD with respiratory disease., Conclusions: Despite a large proportion of individuals at risk for COPD or having COPD and a high prevalence of breathlessness, awareness of respiratory symptoms and knowledge of COPD were limited. These findings indicate a need for educating the general population about COPD., (Copyright © 2011 SPLF. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Chronic obstructive pulmonary disease (COPD) is markedly under-diagnosed, which may relate to under-reporting of symptoms and poor awareness of the disease., Methods: A survey was conducted in a sample of the French general population aged 40-75 years (n=2758) to assess respiratory symptoms and level of knowledge of the disease in subjects with or at-risk of COPD (n=860, 31%)., Results: The high frequency of dyspnoea (MRC dyspnoea grade > or =1: 40%) contrasted with that of subjects spontaneously reporting respiratory problems (9%). Among these, 72% reported limitations in daily-life activities but only 14% considered that they were severely affected by their respiratory status. A very low proportion of subjects knew the term COPD (8%) and only 66% of these associated COPD with a respiratory disease., Conclusions: There is a wide gap between the high number of subjects at risk of COPD in the general population and the frequency of breathlessness in these subjects on one hand, and the poor knowledge of the disease, poor perception of symptoms, and under-diagnosis and under-use of spirometry on the other. Increasing awareness of COPD in the population is needed.