[French practical guidelines for the diagnosis and management of IPF - 2021 update, full version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version intégrale.

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.

[French practical guidelines for the diagnosis and management of IPF - 2021 update, short version]. / Recommandations pratiques pour le diagnostic et la prise en charge de la fibrose pulmonaire idiopathique ­ Actualisation 2021. Version courte.

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.

[The catamenial pneumothorax: a diagnosis often overlooked]. / Le pneumothorax cataménial : un diagnostic souvent méconnu.

The catamenial pneumothorax (CP) is defined as recurrent pneumothorax occurring from the day before menstruations until 72 hours after their beginning, but remains a diagnostic and therapeutic problem. We herein report the cases of two young women who presented several episodes of pneumothorax. The first patient (28 years old) underwent 18 recurrent pneumothorax before the diagnosis of CP. The video-assisted mini-thoracotomy found many diaphragmatic perforations, which were sutured after resection and biopsy. The latter patient underwent four pneumothorax before diagnosis of CP. A video-assisted mini-thoracotomy associated with pleurectomy and pleural freshening was then performed. CP is a rare entity of spontaneous pneumothorax often associated with thoracic endometriosis. The difficulty remains in diagnosis (diagnostic delay before the start of appropriate care), as well as in the treatment. Overall, CP requires a multidisciplinary approach combining pulmonology, thoracic surgery and gynecology.

[Daily physical activity in adult patients with cystic fibrosis]. / Activité physique quotidienne de patients adultes atteints de mucoviscidose.

Assessment of daily physical activity (DPA) is a major element in the development of respiratory rehabilitation. The aim of this study was to evaluate the DPA and the daily energy expenditure (DEE) in adult patients with cystic fibrosis. Thirty adult patients (16 women, 14 men, mean age 27.1±8.4 years) with cystic fibrosis (FVC: 69.2±19%, FEV1: 55.1±24%, BMI: 20.5±3.6kg/m(2)), in stable condition were included in the study. The questionnaire allowed DPA to estimate DEE from the intensity and type of activity. This tool divides the DEE into four intensity levels: less than 1 Metabolic Equivalent Task (MET), greater than 1 and less than 3 METs, 3-5 METs, and greater than 5 METs. A shuttle test for estimating VO(2) peak was performed in all patients. The DEE reached 9478.4±3224.3kJ24h(-1) and was significantly and highly correlated with calculated peak VO(2) (l/min) (r=0.876, P=<0.001); 22% DEE was less than 3 METs, 10% between 3 and 5 METs et 2,5% greater than 5 METs; 65% DEE was less than 1 MET. There was a strong correlation between DEE and BMI (r=0.860, P=<0.001), a low correlation with FVC (r=0.423, P=0.02). This questionnaire is relevant for estimating DPA and DEE in patients with cystic fibrosis and is an interesting test to be used to evaluate changes in patients after pulmonary rehabilitation.

[New toxicity of fotemustine: diffuse interstitial lung disease]. / Nouvelle toxicité de la fotémustine : une pneumopathie interstitielle diffuse.

BACKGROUND: Fotemustine is an alkylating cytostatic drug belonging to the nitrosourea family and is used in particular in the treatment of disseminated malignant melanoma. Herein, we report a case of interstitial lung disease associated with fotemustine. PATIENTS AND METHODS: An 81-year-old man treated with fotemustine for metastatic melanoma presented acute interstitial lung disease 20 days after a fourth course of fotemustine monotherapy. The condition regressed spontaneously, with the patient returning to the clinical, radiological and blood gas status that had preceded fotemustine treatment. After other potential aetiologies had been ruled out, acute fotemustine-induced lung toxicity was considered and this treatment was definitively withdrawn. DISCUSSION: Other cytostatic agents belonging to the nitrosourea family can cause similar pictures, with a number of cases of interstitial lung disease thus being ascribed to fotemustine and dacarbazine. To our knowledge, this is the first case of interstitial lung disease induced by fotemustine monotherapy. This diagnosis should be considered where respiratory signs appear in melanoma patients undergoing fotemustine treatment.

[Prognostic value of clinical exercise testing in idiopathic pulmonary fibrosis]. / Intérêt pronostique de l'exploration fonctionnelle à l'exercice (EFX) au cours de la fibrose pulmonaire idiopathique.

Current guidelines for referring patients with idiopathic pulmonary fibrosis (IPF) for lung transplantation, based on resting parameters, are insufficient to predict 3-year mortality. The aim of this study was to determine the prognostic value of cardio-pulmonary exercise testing (CPET) in patients with IPF. A multicentre retrospective study of 3-year outcome was made on 63 adult patients with IPF who underwent CPET with blood gas analysis. Demographic data, resting pulmonary function and CPET parameters were collected to perform a univariate survival analysis. To estimate prognosis at 3 years, a multivariate logistic regression analysis by Kaplan-Meier curves and log-rank tests was performed. Forty-four patients (70%) were alive without lung transplant at the end of the 3-year follow-up: 19 patients (30%) were dead (n=14) or transplanted (n=5). Univariate analysis indicated that: at rest lower TLC, FVC, DLCO and PaCO(2), higher alveolo-arterial gradient for oxygen [P(A-a)O(2)] and pH; at ventilatory threshold (VT) higher VE/VO(2) and VE/VCO(2) and at peak exercise higher VE/VO(2) and VE/VCO(2), higher pH and ΔP(A-a)O(2)/ΔVO(2) (mmHg/L), lower VO(2) peak, PaO(2) and VO(2)/FC were associated with a significantly lower survival at 3 years. The multivariate logistic regression analysis showed that CPT (<65%) and VE/VO(2) at VT (>45) were independently associated with a lower survival at 3 years. Restriction and hyperventilation at ventilatory threshold are major prognostic factors in the course of IPF. CPET with blood gas analysis may have a prognostic value in these patients and initial evaluation of these parameters can help to predict disease progression.