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International audience; Introduction: Primary spontaneous pneumothorax (PSP) is the presence of air in the pleural space, occurring in the absence of trauma and known lung disease. Standardized expert guidelines on PSP are needed due to the variety of diagnostic methods, therapeutic strategies and medical and surgical disciplines involved in its management. Methods: Literature review, analysis of literature according to the GRADE (Grading of Recommendation Assessment, Development and Evaluation) methodology; proposals for guidelines rated by experts, patients, and organizers to reach a consensus. Only expert opinions with strong agreement were selected. Results: A large PSP is defined as presence of a visible rim along the entire axillary line between the lung margin and the chest wall and superior or equal to 2 cm at the hilum level on frontal chest x-ray. The therapeutic strategy depends on the clinical presentation: emergency needle aspiration for tension PSP; in the absence of signs of severity: conservative management (small PSP), needle aspiration or chest tube drainage (large PSP). Outpatient treatment is possible if a dedicated outpatient care system is previously organized. Indications, surgical procedures and perioperative analgesia are detailed. Associated measures, including smoking cessation, are described. Conclusion: These guidelines are a step towards PSP treatment and follow-up strategy optimization in France.; Introduction : Le pneumothorax spontané primaire (PSP) est un épanchement gazeux dans la cavité pleurale, survenant hors traumatisme et pathologie respiratoire connue. Des recommandations formalisées d'experts sur le sujet sont justifiées par les pluralités de moyens diagnostiques, stratégies thérapeutiques et disciplines médicochirurgicales intervenant dans leur prise en charge. Méthodes : Revue bibliographique, analyse de la littérature selon méthodologie GRADE (Grading of Recommendation Assessment, Development and Evaluation) ; propositions de recommandations cotées par experts, patients et organisateurs pour obtenir un consensus. Seuls les avis d'experts avec accord fort ont été retenus. Résultats : Un décollement sur toute la hauteur de la ligne axillaire et supérieur ou égal à 2 cm au niveau du hile à la radiographie thoracique de face définit la grande abondance. La stratégie thérapeutique dépend de la présentation clinique : exsufflation en urgence pour PSP suffocant ; en l'absence de signe de gravité : prise en charge conservatrice (faible abondance), exsufflation ou drainage (grande abondance). Le traitement ambulatoire est possible si organisation en amont de la filière. Les indications, procédures chirurgicales et l'analgésie périopératoire sont détaillées. Les mesures associées, notamment le sevrage tabagique, sont décrites. Conclusion : Ces recommandations sont une étape de l'optimisation des stratégies de traitement et de suivi des PSP en France.

International audience

International audience

National audience; Systemic sclerosis (SSc) is an autoimmune disease associated to fibrotic manifestations. Interstitial lung disease (SSc-ILD), one of the main fibrotic features of SSc, is the first cause of SSc-related death. The management of SSc-ILD has recently benefited from the results of key randomised controlled trials. French authorities have approved Nintedanib for the treatment of SSc-ILD, and tocilizumab has recently been approved by the Food and Drug Administration (FDA) in the United-States (US). These recent approvals challenge the management of this fibrotic manifestation of SSc. This narrative literature review, at the crossroad of internal medicine and pulmonology, discusses what could be an up-to date approach, in terms of diagnostic and therapeutic strategies for SSc-ILD, in the light of the results from recent clinical trials.

National audience; Hypersensitivity Pneumonitis (HP) is a common immune-mediated interstitial lung disease (ILD) induced by repeated exposure to environmental antigens in susceptible individuals. The most commonly known forms are bird fancier’s disease and farmer’s lung. However, the antigens involved are widely diverse. Therefore, the list of causes of HP is frequently expanding. HP seems to be under-diagnosed owing to its highly heterogeneous presentation in both the non-fibrotic and fibrotic subtypes and could represent up to 15% of all ILDs encountered in clinical practice. However, the recognition of HP cases is essential to ensure appropriate therapy for the patient. Home health care workers’ intervention is sometimes critical in this context. In case of confirmed exposure, the diagnosis could be considered with high confidence if the high-resolution computed tomography (HR-CT) shows a typical HP pattern associated with a lymphocytosis over 30% in the broncho-alveolar lavage (BAL). In all other situations, the patients should undergo further investigations and additional histopathological sampling should be considered and submitted to a multidisciplinary team discussion. After diagnosis, antigenic eviction is the rule whenever possible. Corticosteroid treatment is the first-line medical treatment for severe forms and aims to prevent the development of fibrosis. Anti-fibrotic therapy is now an option for patients with progressive ILD and failure of immunomodulatory/immunosuppressive therapies.

BackgroundGastrointestinal (GI) involvement is a common manifestation of systemic sclerosis (SSc) and a frequent side-effect of drugs used to treat SSc. In the SENSCIS trial, nintedanib reduced the rate of decline in forced vital capacity (FVC) in patients with SSc-associated interstitial lung disease (SSc-ILD), with an adverse event profile characterised predominantly by GI events.ObjectivesTo assess the severity and impact of GI symptoms on quality of life in patients treated with nintedanib in the open-label extension trial, SENSCIS-ON.MethodsPatients with SSc-ILD who completed the SENSCIS trial or a drug–drug interaction (DDI) study of nintedanib and oral contraceptive were eligible to enter SENSCIS-ON. Patients who received nintedanib in SENSCIS (up to 100 weeks) and continued nintedanib in SENSCIS-ON comprised the “continued nintedanib” group. Patients who received placebo in SENSCIS and initiated nintedanib in SENSCIS-ON, or who received nintedanib for a short time in the DDI study, comprised the “initiated nintedanib” group. We assessed changes in scores on the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract (UCLA SCTC GIT) questionnaire v2.0 from baseline to week 52. This questionnaire comprises 7 scales measuring the severity and impact of GI symptoms: reflux, distension or bloating, faecal soilage, diarrhoea, constipation, emotional well-being, social functioning. Each scale is scored from 0 to 3 except for the diarrhoea scale (0 to 2) and constipation scale (0 to 2.5). The total score, the mean of the scores for the scales except constipation, ranges from 0 to 2.83, with higher scores indicating worse symptoms.ResultsThe “continued nintedanib” group comprised 197 patients and the “initiated nintedanib” group comprised 247 patients (231 from SENSCIS). Of these, 178 and 218 patients, respectively, provided a total UCLA SCTC GIT score at baseline. At baseline, mean (SD) total scores were 0.33 (0.33) and 0.33 (0.34) in the continued nintedanib and initiated nintedanib groups, respectively. Mean (SD) scores on the 7 scales ranged from 0.16 (0.52) to 0.70 (0.73) in the continued nintedanib group and from 0.13 (0.43) to 0.64 (0.68) in the initiated nintedanib group. Increases (worsening) in scores were observed in both groups from baseline to week 52, except for on the constipation scale (Figure 1). Based on the total score, between baseline and week 52, the proportion of patients with moderate or severe or very severe GI symptoms increased, but 45.7% and 39.7% of patients in the continued nintedanib and initiated nintedanib groups, respectively, had no or mild GI symptoms at week 52 (Table 1).Table 1.Changes in severity and impact of gastrointestinal symptoms based on UCLA SCTC GIT total score between baseline and week 52 of SENSCIS-ONBaselineWeek 52None or mildModerateSevere or very severeMissingTotalContinued nintedanibNone or mild81 (41.1)5 (2.5)04 (2.0)90 (45.7)Moderate38 (19.3)10 (5.1)01 (0.5)49 (24.9)Severe or very severe13 (6.6)14 (7.1)7 (3.6)1 (0.5)35 (17.8)Missing6 (3.0)3 (1.5)1 (0.5)13 (6.6)23 (11.7)Total138 (70.1)32 (16.2)8 (4.1)19 (9.6)197 (100)Initiated nintedanibNone or mild87 (35.2)6 (2.4)1 (0.4)4 (1.6)98 (39.7)Moderate35 (14.2)12 (4.9)2 (0.8)3 (1.2)52 (21.1)Severe or very severe8 (3.2)7 (2.8)4 (1.6)1 (0.4)20 (8.1)Missing37 (15.0)15 (6.1)4 (1.6)21 (8.5)77 (31.2)Total167 (67.6)40 (16.2)11 (4.5)29 (11.7)247 (100)Data are n (%) of patients. None or mild=scores of 0 to 0.49; moderate=scores of 0.5 to 1; severe or very severe=scores of 1.01 to 3.Figure 1.Changes in UCLA SCTC GIT scores from baseline to week 52 of SENSCIS-ONConclusionIn the SENSCIS-ON trial, the majority of patients with SSc-ILD treated with nintedanib had no or mild GI symptoms at baseline. A small worsening in GI symptoms was observed over 52 weeks. Diarrhoea had the greatest impact, reflecting the adverse event profile of nintedanib. Recommendations for the management of diarrhoea in patients treated with nintedanib should be implemented in clinical practice.AcknowledgementsThe SENSCIS-ON trial was funded by Boehringer Ingelheim.Disclosure of InterestsDinesh Khanna Shareholder of: Eicos Sciences, Inc - stocks, Consultant of: AbbVie, Acceleron, Actelion, Amgen, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, Galapagos NV, Genentech/Roche, Gilead, GlaxoSmithKline, Horizon Therapeutics, Merck Sharp & Dohme, Mitsubishi Tanabe Pharma, Sanofi-Aventis, United Therapeutics, Prometheus, Theraly, AstraZeneca, Grant/research support from: Bayer, Bristol-Myers Squibb, Horizon Therapeutics, Immune Tolerance Network, National Institutes of Health, Pfizer, Employee of: CiviBioPharma/Eicos Sciences, Inc - Leadership/Equity position – Chief Medical Officer, Elizabeth Volkmann Speakers bureau: Boehringer Ingelheim, Consultant of: Boehringer Ingelheim, Grant/research support from: Boehringer Ingelheim, Corbus, Forbius, Horizon, Kadmon, Kristin Highland Speakers bureau: Actelion Pharmaceuticals (Jansen), Bayer Healthcare, Boehringer Ingelheim, United Therapeutics, Paid instructor for: Acceleron Pharmaceuticals, Actelion Pharmaceuticals, Bayer Healthcare, Boehringer Ingelheim, Gilead Sciences, United Therapeutics, Consultant of: Boehringer Ingelheim, United Therapeutics, Genentech, Forsee Pharmaceuticals, Grant/research support from: Acceleron Pharmaceuticals, Actelion Pharmaceuticals, Bayer Healthcare, Boehringer Ingelheim, Genentech, Gossamer Bio, Eiger Pharmaceuticlas, Lilly Pharmaceuticals, Reata Pharmaceuticals, United Therapeutics, Viela Bio (Horizon Pharmaceuticals), Yannick Allanore Consultant of: Abbvie, AstraZeneca, Bayer, Boehringer Ingelheim, Janssen, Medsenic, Mylan, Prometheus, Roche, Sanofi, Grant/research support from: Alpine Immunosciences, Medsenic, OSE Immunotherapeutics, Stéphane Jouneau Paid instructor for: Cours, formations - Actelion, AIRB, AstraZeneca, Bristol-Myers Squibb, Boehringer Ingelheim, Chiesi, GlaxoSmithKline, LVL, Mundipharma, Novartis, Pfizer, Roche, Consultant of: Advisory Boards, consultancy - AIRB, Boehringer Ingelheim, Roche, Grant/research support from: Recherche Clinique - AIRB, Biogen, Bristol-Myers Squibb, Boehringer Ingelheim, Galactic, Gilead, LVL, Roche, SavaraAides pour des recherches - AIRB, Boehringer Ingelheim, LVL, Novartis, Roche, James Seibold Shareholder of: Prometheus Biosciences, Speakers bureau: Boehringer Ingelheim, Consultant of: Alexion, Blade, Camurus AB, GlaxoSmithKline, Prometheus Biosciences, Sironax, Sojournix, Xenikos, Employee of: Prometheus Biosciences, Alexandra James Employee of: Alexandra James is an employee of Elderbrook solutions GmbH that is contracted by Boehringer Ingelheim, Margarida Alves Employee of: Margarida Alves is an employee of Boehringer Ingelheim, Oliver Distler Speakers bureau: OD has/had relationships with the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years:Speaker fee: Bayer, Boehringer Ingelheim, Janssen, Medscape, Consultant of: OD has/had relationships with the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years:Consultancy fee: Abbvie, Acceleron, Alcimed, Amgen, AnaMar, Arxx, AstraZeneca, Baecon, Blade, Bayer, Boehringer Ingelheim, Corbus, CSL Behring, 4P Science, Galapagos, Glenmark, Horizon, Inventiva, Kymera, Lupin, Miltenyi Biotec, Mitsubishi Tanabe, MSD, Novartis, Prometheus, Roivant, Sanofi and TopadurOD has/had relationships with the following companies in the area of potential treatments for arthritides in the last three calendar years:Consultancy fee: Abbvie, Grant/research support from: OD has/had relationships with the following companies in the area of potential treatments for systemic sclerosis and its complications in the last three calendar years:Research Grants: Boehringer Ingelheim, Kymera, Mitsubishi Tanabe

Introduction La maladie a nouveau coronavirus 2019 (COVID-19) a actuellement un taux de progression rapide mondial. Le nombre de cas severes rapportes chez les greffes d’organes solides reste limite, mais leur statut d’immunodeprime fait supposer un risque eleve de complications liees au SARS-Cov-2. L’insuffisance respiratoire aigue liee au syndrome de detresse respiratoire aigue (SDRA), qui se developpe chez environ 5 % des patients, reste la cause principale de deces. Il est maintenant etabli qu’un sous-groupe de patients porteurs d’infection severe COVID-19 developpe un syndrome de tempete cytokinique associe au risque de deces, chez qui des traitements immunomodulateurs pourraient etre benefiques. Les options therapeutiques, entre autres, incluent les corticoides a doses elevees, mais il etait anticipe une balance risque–benefice defavorable a leur utilisation au tout debut de l’epidemie. Une autre question dans la population des transplantes pulmonaires (TxP) est l’eventuel role prophylactique de l’azithromycine au long cours sur l’acquisition du SARS-Cov-2, frequemment administre dans cette population specifique en cas de la dysfonction chronique du greffon. Resultats Nous rapportons 2 cas severes de SDRA-COVID-19 hyper-inflammatoires ayant menace leur pronostic vital, survenus au sein de notre cohorte monocentrique de greffes pulmonaires malgre un traitement d’azithromycine faible-dose au long cours. Un traitement par methylprednisone a doses elevees (2 mg/kg/j), debute precocement apres le debut du SDRA sous ventilation mecanique, a permis une recuperation pulmonaire rapide et ad integrum chez les 2 patients. Conclusion Ces 2 cas de SDRA-COVID-19 suggerent : – que des formes graves peuvent survenir malgre un traitement au long-court d’azithromycine faible-dose apres TxP ; – le benefice de l’utilisation precoce des corticoides a dose elevee en cas de survenue de SDRA-COVID-19 hyper-inflammatoire apres TxP, comme observe recemment dans un essai therapeutique testant l’efficacite de la dexamethasone au sein d’une population generale de patients.

Introduction Le programme OPALE a pour objectif d’apporter un soutien aux patients atteints de FPI et traites par nintedanib ou pirfenidone. Ces traitements, souvent associes a des evenements indesirables, peuvent impacter la qualite de vie des patients et l’adherence au traitement. Le programme OPALE consiste en un suivi telephonique regulier au cours des 9 premiers mois de traitement et la possibilite de joindre un numero vert pour repondre a des questions eventuelles. Methodes Les appels par une infirmiere specialisee ont lieu des l’initiation du traitement (j0), et a intervalles reguliers : j + 15, puis a 2, 3 et 9 mois. L’appel consiste en un soutien et permet d’evaluer l’observance grâce a un questionnaire CNAM. Un compte rendu est aussi mis a disposition du pneumologue. Les patients completent des auto-questionnaires papiers a j0, M3 et M9 : (DIRECT : Disability RElated to COPD Tool ; CAT : COPD Assessment Tool ; HAD Hospital Anxiety and Depression Scale). Resultats Depuis octobre 2015, 298 ont termine le programme OPALE sur 383 patients inscrits. Parmi ces patients, 171 (57 %) ont complete leur suivi sur les 9 mois sans arret de traitement. Les raisons pour lesquelles les patients n’ont pas complete leur suivi sont le deces (n = 10), l’abandon du programme (n = 40) et l’arret du traitement a cause des evenements indesirables (n = 53) ou pour d’autres motifs (hospitalisation…) (n = 24). Les patients avaient un âge moyen de 72,8 ans ( ± 8,7) avec une predominance masculine (80 %). Quatre-vingt-seize pour cent sont en initiation de traitement majoritairement traites par nintedamib (83 %). L’observance etait stable pour les 171 patients suivis pendant 9 mois, avec un score CNAM moyen de 5,76 ( ± 0,67)/6 a j15 et 5,64 (±0,85)/6 a M9. Cent trente-trois (44 %) patients ont envoye leur questionnaire a j0 ; 62 (20 %) a M3 et 31 (10 %) a M9. Les scores moyens entre j0 et M9 ne sont pas differents ( Tableau 1 ). 215 (72 %) patients ont appele au moins une fois le numero vert et 101 (33 %) patients l’ont appele trois fois ou plus. La duree moyenne totale de communication par patient de 20 minutes, suggerant un gain probable de temps pour les equipes soignantes des patients inclus dans OPALE ( Tableau 1 ). Conclusion Depuis 2015, 78 % des 383 inscrits ont suivi le programme d’accompagnement pendant 9 mois en maintenant une observance satisfaisante. Le numero vert permet aux patients de poser leurs questions aux infirmieres specialisees. Dans l’ensemble, la qualite des patients evaluee par des echelles HAD, CAT et DIRECT entre j0 et M9 est stabilisee.

Pulmonary metastases due to a pancreatic cancer are difficult to diagnose and demonstrate a wide range of radiological patterns. We report the case of a 37-year-old female patient, without past medical history, with multicystic lung disease in a context of chronic abdominal pain, fatigue and weight loss. After several months of diagnostic delay, pathological examination of surgical lung biopsies led to the diagnosis of secondary deposits of pancreatic cancer. The clinical and radiogical situation deteriorated quickly with the development of alveolar consolidation and Aspergillus superinfection was then diagnosed. This case illustrates the value of an early decision to undertake surgical lung biopsy in the work-up of multicystic lung disease when cancer is suspected. In addition, in the specific context of cancer, faced with clinical and/or radiological deterioration, it is essential to look for infection, particularly aspergillosis.

Lung volume reduction can be effective in appropriately selected patients with severe emphysema and is associated with reduced breathlessness and improved survival. Spontaneous resolution of emphysematous bullae can also sometimes occur.We report a case of severe smoking-related emphysema in a 60-year-old patient, who presented in October 2013 with a right upper lobe acute community-acquired pneumonia on the background of previously undocumented emphysema. The patient improved following treatment with co-amoxiclav and serial radiology showed progressive cicatricial retraction. Nine months later there had been a major functional improvement characterized by a complete normalization of the patient's ventilatory parameters, specifically a 45% improvement in FEVRarely, emphysematous bullae resolve following infections. Further studies of the mechanisms involved in these natural regressions may be of interest in the development of new therapeutics.

International audience; no abstract

Hypersensitivity pneumonitis (HP) are typically subacute in their presentation and the diagnosis may be difficult.We report a case of a hypersensitivity pneumonitis in a 27-years-old woman, caused by exposure to mould in an insalubrious mobile home. The initial presentation was with acute respiratory distress syndrome complicating RSV pneumonia, treated with ribavirin and corticosteroids in winter 2013-2014. The diagnosis of hypersensitivity pneumonitis was based on clinical and radiological relapse occurring during winter 2014-2015 with confirmed exposure to antigen with fungal sampling at home, respiratory deterioration with antigen rechallenge and a compatible chest CT-scan.The diagnosis of hypersensitivity pneumonitis should be considered in similar cases. Treatment is based mainly on removing exposure to the causative antigen.

The aim of our study was to compare the features at diagnosis in patients with ornithosis to patients with avian acute hypersensitivity pneumonitis (HP). Clinical, biological and radiological differences could potentially help clinicians to distinguish these diseases.We conducted a retrospective study on patients admitted from 2000 to 2016 in three hospitals. Ornithosis was diagnosed based on a positive polymerase chain reaction for Chlamydophila psittaci on respiratory samples and/or a seroconversion while HP was diagnosed on the basis of at least one positive serum precipitin.Twelve patients with HP and 13 patients with ornithosis were identified. Compared to HP, ornithosis occurred more frequently in males (P=0.047), with less previous respiratory diseases (P=0.01), shorter symptom duration (P=0.03), less frequently bilateral crackles (P=0.004), more severe disease requiring more frequently intensive care admission (P=0.005), higher CRP values (P=0.005) and more profound lymphopenia (P=0.02). Ground glass shadowing on CT scan (P=0.001) or bronchiectasis (P=0.03) were more frequently noted in patients with HP.Our results suggest that patients with ornithosis and HP have important differences in their clinical, biological, and radiological presentation.

Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis and detecting PAH efficiently remains challenging. The DETECT study has offered in 2013 a composite screening tool for PAH. The objective of our study was to compare the indication of right heart catheterisation (RHC) as suggested by the DETECT algorithm with the decisions of a multidisciplinary team.This prospective monocentric non-interventional study consecutively included systemic sclerosis patients when data required to apply DETECT algorithm were available. We evaluate the number of RHC as requested by this algorithm and confronted it with the indications of RHC suggested by a multidisciplinary group blinded for the result of DETECT algorithm.In total, 117 systemic sclerosis patients were included. When DETECT algorithm was applied to all patients, RHC was suggested by this algorithm for 70 patients, whereas only 15 indications were required by the multidisciplinary group; among those patients only 7 had PAH. When DETECT algorithm was applied only to the 42 patients with DLCO60% and disease duration of more than 3 years, RHC was suggested for 31 patients whereas only 13 were indicated by the multidisciplinary group; among those patients only 7 had PAH.The DETECT algorithm is able to efficiently detect all PAH patients finally diagnosed by our multidisciplinary team. However, it increases by 3 the number of RHC that should be performed.

Nocardiosis is an infectious disease with wide range of clinical features, which can eventually lead to death. The agent responsible belongs to the genus Nocardia that includes about fifty different species. Nocardiosis occurs mainly in immunocompromised hosts. We report here three cases of disseminated nocardiosis misdiagnosed initially as cerebral metastatic lung cancer. These patients, including two immunocompetent hosts, presented with both pulmonary and cerebral lesions. In all three patients, the diagnosis was based on magnetic resonance imaging with diffusion sequence, apparent diffusion coefficient reconstruction and neurosurgical cerebral biopsies. Treatment with an appropriate antibiotic regimen was prolonged for several months. Progress was favorable with full resolution of the neurological symptoms and the radiological abnormalities. These three cases emphasize the diagnostic challenge of nocardiosis, especially in disseminated disease.

Pancreatic cancer is often not diagnosed until at a metastatic stage at which point the prognosis is very poor. Pulmonary metastases are pleomorphic, often present at the time of diagnosis and can lead to the discovery of an asymptomatic primary disease.We describe two cases aged 60 and 74 years, where imaging identified what was thought to be an interstitial lung disease but which was actually metastasis from pancreatic cancer. In the first case, CT showed multiple excavated pulmonary nodules but the presentation with medullary compression led rapidly to pathological diagnosis on bone lesions. In the second patient, a history of rheumatoid arthritis and the lack of abdominal symptoms led to an initial search for disease related to the rheumatoid disease. Histopathology, from lung and bone biopsies, enabled a correct diagnosis to be achieved.Where atypical interstitial lung disease occurs, biopsy should be considered in order not to delay a diagnosis of cancer, especially pancreatic cancer.

Resume Les nouvelles recommandations pour la prise en charge de la fibrose pulmonaire idiopathique (FPI) seront publiees prochainement. La FPI est une pneumopathie interstitielle chronique fibrosante de l’adulte d’etiologie inconnue et dont l’histoire naturelle est variable et imprevisible. Son diagnostic repose, tout d’abord, sur l’exclusion de toute autre cause de pneumopathie interstitielle (exposition environnementale, medicaments, connectivite). Il est fonde, sur l’association d’une presentation clinique evocatrice et d’un aspect tomodensitometrique et/ou anatomopathologique de pneumopathie interstitielle commune (PIC) ou « usual interstitial pneumonia » (UIP). Il necessite le plus souvent une concertation multidisciplinaire entre pneumologues, radiologues et anatomopathologistes.