Objectives: To analyse functional outcome parameters according to antimicrobial treatments after respiratory syncytial virus (RSV)-confirmed infection in adult lung transplant recipients. Methods: A 9-year retrospective multicentre cohort study (2011-19) included adult lung transplant recipients with RSV-confirmed infection. The first endpoint determined new allograft dysfunction (acute graft rejection and chronic lung allograft dysfunction (CLAD)) 3 months after infection. Then baseline and 3 months' postinfection forced expiratory volume in 1 second (FEV1) values were compared according to antimicrobial treatment. Univariate logistic regression analysis was performed. Results: RSV infection was confirmed in 77 of 424 lung transplant recipients (estimated incidence of 0.025 per patient per year; 95% confidence interval 0.018-0.036). At 3 months, 22 recipients (28.8%) developed allograft dysfunction: ten (13%) possible CLAD, six (7.9%) acute rejection and six (7.9%) CLAD. Recipients with the lowest preinfection FEV1 had a greater risk of developing pneumonia (median (interquartile range) 1.5 (1.1-1.9) vs. 2.2 (1.5-2.4) L/s, p 0.003) and a higher odds of receiving antibiotics (1.6 (1.3-2.3) vs. 2.3 (1.9-2.5) L/s, p 0.017; odds ratio 0.52, 95% confidence interval 0.27-0.99). Compared to tracheobronchitis/bronchiolitis, RSV-induced pneumonia led more frequently to hospitalization (91.7%, 22 vs. 58.0%, 29, p 0.003) and intensive care unit admission (33.3%, 8 vs. 0, p < 10(-3)). For ribavirin-treated recipients (24.7%, 19) and azithromycin prophylaxis (50.6%, 39), 3-month FEV1 values were not different from untreated recipients. The overall mortality was 2.5% at 1 month and 5.3% at 6 months, unrelated to RSV. Conclusions: At 3 months after RSV-confirmed infection, 22 recipients (28.8%) had new allograft dysfunction. Ribavirin treatment and azithromycin prophylaxis did not prevent FEV1 decline. (C) 2020 European Society of Clinical Microbiology and Infectious Diseases. Published by Elsevier Ltd. All rights reserved.

Abstract Background Pleuroparenchymal fibroelastosis (PPFE) has a variable disease course with dismal prognosis in the majority of patients with no validated drug therapy. This study is to evaluate the effect of nintedanib in patients with idiopathic and secondary PPFE. Patients admitted to a tertiary care center (2010–2019) were included into this retrospective analysis if they had a multidisciplinary diagnosis of PPFE, had been followed-up for 3 months or more, and had lung function tests and chest CTs available for review. Changes in pulmonary function tests were assessed using non-parametric tests and linear mixed effect model. Lung volumes were measured with lobar segmentation using chest CT. Results Out of 21 patients with PPFE, nine had received nintedanib, six had received another treatment and another six patients were monitored without drug therapy. Annual FVC (% of predicted) relative decline was − 13.6 ± 13.4%/year before nintedanib and − 1.6 ± 6.02%/year during nintedanib treatment (p = 0.014), whereas no significant change in FVC% relative decline was found in patients receiving another treatment (− 13.25 ± 34 before vs − 16.61 ± 36.2%/year during treatment; p = 0.343). Using linear mixed effect model, the slope in FVC was − 0.97%/month (95% CI: − 1.42; − 0.52) before treatment and − 0.50%/month (95% CI: − 0.88; 0.13) on nintedanib, with a difference between groups of + 0.47%/month (95% CI: 0.16; 0.78), p = 0.004. The decline in the upper lung volumes measured by CT was − 233 mL/year ± 387 mL/year before nintedanib and − 149 mL/year ± 173 mL/year on nintedanib (p = 0.327). Nintedanib tolerability was unremarkable. Conclusion In patients with PPFE, nintedanib treatment might be associated with slower decline in lung function, paving the way for prospective, controlled studies.

Pulmonary fibrosis is considered to result from recurrent alveolar epithelial injury coupled with dysfunctional alveolar wound healing mechanisms, some of which have a genetic background. Pulmonary fibrosis in the adult has not been previously associated with prolidase deficiency, an innate deficiency of amino acid metabolism. Prolidase deficiency is a new genetic cause of combined pulmonary fibrosis and emphysema syndrome in the adult We thank Sylvie Boyer (Lyon) for aminoacids measurement, and Kais Ahmad, Jade Cuilleron, Sylvie Ernesto, Laure Folliet, Nathalie Guffon, Lize Kiakouama, Jacques-Olivier Maillard, Pascale Nesme-Meyer and Judith Proovost and for clinical care of the patient.

La fibroélastose pleuroparenchymateuse (FEPP) est une maladie pulmonaire fibrosante évolutive souvent létale, caractérisée par un dépôt de fibres élastiques au niveau du parenchyme pulmonaire et de la plèvre, prédominant dans les parties supérieures du thorax [1]. Aucun médicament n’a démontré son efficacité. Nous avons souhaité évaluer si un traitement anti-fibrosant utilisé dans la fibrose pulmonaire idiopathique pourrait réduire la progression de la FEPP. Nous rapportons notre expérience de l’utilisation du nintédanib, un inhibiteur intracellulaire ciblant plusieurs tyrosine kinases, associé à une corticothérapie à dose moyenne, chez les patients ayant une FEPP d’évolution progressive. Nous avons revu rétrospectivement les dossiers de tous les patients diagnostiqués avec FEPP et admis dans le un Centre de référence des maladies pulmonaires rares entre janvier 2010 et décembre 2018. Les patients étaient traités à la discrétion du médecin après discussion multidisciplinaire ; les traitements étaient prescrits hors AMM. Sur 21 patients atteints de FEPP (dont 17 idiopathiques), 11 ont reçu du nintédanib (150 mg deux fois par jour) pendant au moins 3 mois, et 9 patients (dont 7 idiopathiques) ayant un suivi d’au moins 6 mois ont été inclus dans l’analyse. Un aspect histologique et/ou tomodensitométrique de pneumopathie interstitielle commune certaine ou probable était présent chez 3/9 malades traités. Le nintédanib a été associé à 10 à 15 mg/j de prednisone chez 6 patients. Le déclin annuel de la capacité vitale forcée (CVF) a été évalué avant et sous nintédanib. Chez les 9 patients traités, la CVF a diminué régulièrement avant l’initiation du nintédanib (déclin moyen de 274 ± 188 mL/an). Après un suivi médian de 372 jours, le traitement par le nintédanib a été associé à une diminution de la pente du déclin de la CVF (169 ± 214 mL/an ; p = 0,023). La tolérance du nintédanib était généralement bonne malgré un poids corporel moyen relativement faible (48,9 ± 8,7 kg). Le nintédanib pourrait réduire la vitesse de déclin de la CVF chez les patients atteints de FEPP idiopathique ou secondaire. Un essai thérapeutique randomisé est nécessaire pour évaluer le bénéfice et la tolérance de ce traitement au cours de cette affection grave.

Background: After lung transplantation (LT), between 2% and 25% of bronchial anastomoses develop complications requiring therapeutic intervention. The status of healing of both bronchial anastomoses and downhill airways are well described by the French consensual MDS standardized grading system (Macroscopic, Diameter, Suture). We analyzed risks factors for airway complications (AC) after transplantation and the way we managed them. We report here our challenging method of early rigid bronchoscopic intervention with airway stenting on bronchial healing. Methods: All single center consecutives LTs were retrospectively analyzed between 2010-2016. Patient-level data (demographic, peri-operative data) and anastomosis-level data (surgical parameters, bronchoscopy findings) were monitored. The incidence and contributive factors of ACs are reported. We also reported modalities of the conservative treatment and outcome. Results: A total of 121 LTs were performed, 39 single-lung and 82 bilateral sequential LT. Main indication for LT were cystic fibrosis (45%) and emphysema (25%) and 58 were male patients (n=70). After a waiting period of healing, 28 patients presented AC on 41 anastomoses (prevalence: 23%). A multivariate analysis found as contributive factors of ACs, post-operative infection by Aspergillus [odds ratio (OR) 2.7, 95% confidence interval (CI): 1.08-6.75; P=0.033] at the patient level, and at the anastomasis level, emphysema (OR 2.4, 95% CI: 1.02-5.6; P=0.045), early dehiscence (OR 11.2, 95% CI: 1.7-76; P=0.01) and cold ischemia time >264 min (OR 2.45, 95% CI: 1.08-5.6; P=0.03). All the 41 ACs were managed conservatively with rigid bronchoscopy (range, 1-10), 41 stents (21 in silicone and 20 fully-covered Silicone Expandable Metallic Stents) without major complication. 717wo AC were still under regular bronchoscopic care and silicone stenting for long left bronchus reason. No surgical intervention was needed. The 2-years overall survival rate where not different between AC group and controls, respectively 85% and 81%. Conclusions: Airway healing after transplantation remains a scalable process and the French consensual MDS classification helped us for therapeutic decisions. Rigid bronchoscopy and safety use of current stenting devices may have the pivotal role in the conservative management of ACs, avoiding perilous situation of surgery for AC. Despite a high rate of AC, their favorable evolution may be explained by the cautious care of airway healing and maybe by the use of the Celsior antioxidant solution.

BACKGROUND:Chronic kidney disease (CKD) after lung transplantation (LT) is underestimated. The aim of the present study was to measure the loss of glomerular filtration rate (GFR) 1 year after LT and to identify the risk factors for developing Stage ≥3 CKD. METHODS:LT patients in the University Hospital of Lyon had a pre- and post-transplantation measurement of their GFR (mGFR), and GFR was also estimated using the Chronic Kidney Disease Epidemiology Collaboration equation. RESULTS:During the study period, 111 patients were lung transplant candidates, of which 91 had a pre-transplantation mGFR, and 29 had a mGFR at 1 year after LT. Six patients underwent maintenance haemodialysis after transplantation. Mean mGFR was 106 mL/min/1.73 m2 before LT and 58 mL/min/1.73 m2 1 year after LT (P < 0.05) with a mean loss of 48 mL/min/1.73 m2 per patient. The risk of developing Stage ≥3 CKD after LT was higher in patients with lower pre-LT mGFR (odds ratio for each 1 mL/min/1.73 m2 increase: 0.94, 95% confidence interval 0.88-0.99). Receiver operator characteristics curves for the sensitivity and specificity of eGFR and mGFR for the prediction of CKD Stage ≥3 after LT found that pre-LT mGFR of 101 mL/min/1.73 m2 and pre-LT eGFR of 124 mL/min/1.73 m2 were the optimal thresholds for predicting Stage ≥3 CKD after LT. CONCLUSION:The present study underlines the value of mGFR in the pre-LT stage and found major renal function loss after LT, and consequently two-thirds of patients have Stage ≥3 CKD at 1 year. All patients with a pre-LT mGFR <90 mL/min/1.73 m2 warrant particular attention.

Alveolar echinococcosis is a rare but life-threatening infection caused by the parasiteEchinococcus multilocularis. Its natural history is characterized by a slow parasitic growth over several years. Increased incidence and shorter development delay have been reported in immune-compromised patients. We report the reactivation of aborted lesions within 12 months of lung transplantation leading to a fast-growing aggressive hepatic lesion. Timely identification of alveolar echninococcosis allowed prompt albendazole treatment and radical surgery leading to a favorable outcome 42 months after transplantation. However, close clinical, serological and radiological monitoring is required to rule out relapses in the long term. The pre-existence of aborted self-limited lesions of alveolar echinococcosis and the possibility for their atypical rapid growth in patients undergoing profound immunosuppression should be known by healthcare providers, even if working in non-endemic areas.

OBJECTIVES: Pathological gastroesophageal reflux (GER) is a known risk factor for bronchiolitis obliterans syndrome (BOS) after lung transplantation. This study aimed at determining whether functional esophageal evaluation might predict BOS occurrence and survival in this setting. METHODS: Ninety-three patients who underwent esophageal high-resolution manometry and 24-hour pH-impedance monitoring within the first year after lung transplantation were retrospectively included. A univariable analysis was performed to evaluate the parameters associated with GER disease and BOS occurrence. The Cox regression model was used to identify the prognostic factors of death or retransplantation. RESULTS: Thirteen percent of patients exhibited major esophageal motility disorders and 20% pathological GER. GER occurrence was associated with younger age, cystic fibrosis, and hypotensive esophagogastric junction. Within a median follow-up of 62 months, 10 patients (11%) developed BOS, and no predictive factors were identified. At the end of the follow-up, 10 patients died and 1 underwent retransplantation. The 5-year cumulative survival rate without retransplantation was lower in patients with major esophageal motility disorders compared with that in those without (75% vs 90%, P=0.01) and in patients who developed BOS compared with that in those without (66% vs 91%; P = 0.005). However, in multivariable analysis, major esophageal motility disorders and BOS were no longer significant predictors of survival without retransplantation. DISCUSSION: Major esophageal motility disorders and BOS were associated with allograft survival in lung transplantation in the univariable analysis. Although the causes of this association remain to be determined, this observation confirms that esophageal motor dysfunction should be evaluated in the context of lung transplantation.

INTRODUCTION:In 2015, the International Society for Heart and Lung Transplantation (ISHLT) published a consensus document for the selection of lung transplant candidates. In the absence of recent French recommendations, this guideline is useful in order to send lung transplant candidates to the transplantation centers and to list them for lung transplantation at the right time. BACKGROUND:The main indications for lung transplantation in adults are COPD and emphysema, idiopathic pulmonary fibrosis and interstitial diseases, cystic fibrosis and pulmonary arterial hypertension (PAH). The specific indications for each underlying disease as well as the general contraindications have been reviewed in 2015 by the ISHLT. For cystic fibrosis, the main factors are forced expiratory volume in one second, 6-MWD, PAH and clinical deterioration characterized by increased frequency of exacerbations; for emphysema progressive disease, the BODE score, hypercapnia and FEV1; for PAH progressive disease or the need of specific intravenous therapy and NYHA classification. Finally, the diagnosis of fibrosing interstitial lung disease is usually a sufficient indication for lung transplantation assessment. OUTLOOK AND CONCLUSION:These new recommendations, close to French practices, help clinicians to find the right time for referral of patients to transplantation centers. This is crucial for the prognosis of lung transplantation.

OBJECTIVE:To describe the prevalence of cystic fibrosis-related diabetes (CFRD) before and after lung transplantation (LT); to analyse the survival and renal function after LT according to the CFRD status before LT. METHODS:Sixty cystic fibrosis (CF) patients transplanted at the Lyon University Hospital between 2004 and 2014 were included. Genotype, pancreatic status, age at LT, survival were recorded. Glucose tolerance status, daily insulin dose requirement, glomerular filtration rate (GFR), and daily glucocorticoid (GC) dose were recorded before LT and until December 2016. RESULTS:The median follow-up was 5.6 (3.8-8.2) years, and nine patients died. Survival was poorest for patients with CFRD before LT compared with those without CFRD (P = 0.03) but was not correlated with the GFR before LT, with sex, age at LT, or CF genotype. The prevalence of CFRD was 68% at 2 years and 54% at 5 years. For persistent insulin-treated CFRD, the insulin requirement decreased (-2.1 IU/d/y; P < 0.01) and was correlated with the daily GC dose (+0.4 IU/d for one additional milligram, P = 0.012). Seven (11%) patients who had insulin-treated CFRD before LT became nondiabetic after LT, with a median time of 2 (1-4) years. After LT, the GFR decreased (-5.3 ml/min/1.73 m 2 /y; P < 0.001) and was not correlated with the CFRD status before LT. CONCLUSIONS:CFRD before LT is associated with poor survival after LT, which should lead to better management of diabetes. Some patients with pre-LT CFRD became nondiabetic after LT. CFRD is not associated with renal insufficiency after LT.

Right heart catheterization (RHC) is the reference test in diagnosing pulmonary hypertension (PH). The increasing age of patients at the time of diagnosis raises the issue of the morbidity of this invasive test in elderly individuals. We hypothesized that the morbidity associated with RHC would be increased in elderly patients and highlight differences in hemodynamic characteristics compared to younger patients. A retrospective study was conducted in a regional referral center for PH. Data for all consecutive RHCs performed during the study period were analyzed. Over a five-year period, 1060 RHCs were performed. Of the patients, 228 (21.5%) were aged >= 75 years and 832 (78.5%) were aged <75 years. Duration of the procedure and site of puncture did not differ according to age group (all P > 0.05). Nine procedures (0.9%) led to complications: three (1.3%) in patients aged >75 years and six (0.7%) in younger patients aged (P = 0.5). Eight were local vascular injuries, directly related to a femoral vein puncture (P < 0.001). Pulmonary arterial pressure and cardiac output were lower in patients aged >75 years than in younger patients (P = 0.001). RHC may be performed regardless of patient age. The rate of RHC complications is not increased in individuals aged >75 years. As most complications were related to femoral vein puncture, this route should be avoided whenever possible.

Group 3 pulmonary hypertension (PH) is a common complication of advanced chronic lung disease. Our hypothesis was that group 3 PH is associated with a more severe baseline presentation and a more severe prognosis compared to group 1 pulmonary arterial hypertension (PAH), chronic thromboembolic PH (group 4), and group 5 PH. We retrospectively analyzed consecutive incident PH patients in a single center between January 2006 and November 2014. Data were acquired from a prospective database. Clinical, functional, and hemodynamic characteristics, as well as survival, were compared between the four groups of precapillary PH. A total of 363 patients were analyzed; 164 patients (45.2%) belonged to group 1 PAH, 109 (30%) to group 3 PH, 65 (17.9%) to group 4 PH, and 25 (6.9%) to group 5 PH. Group 3 patients were predominantly male and were more frequently in New York Heart Association (NYHA) class III/IV. Patients with group 3 and 4 PH were older, had significantly lower 6-min walking distance (6MWD), higher mean pulmonary arterial pressure, higher pulmonary vascular resistance (PVR), and lower cardiac index (CI) than PAH patients. Group 3 and 5 patients had significantly lower total lung capacity (TLC), forced vital capacity (FVC), and FEV1; group 3 patients had the lowest carbon monoxide transfer coefficient values. PH therapy was used in 90.9% of group 3 patients. Univariate analysis of prognostic factors in the overall population showed that age, male gender, NYHA class, groups 3 and 4 PH (vs. PAH), 6MWD, FVC, TLC, carbon monoxide transfer coefficient (KCO), PVR, CI, and venous oxygen saturation were significantly associated with greater mortality. Multivariate analysis showed that age, PH group 4, 6MWD, and KCO but no longer PH group 3 were significantly associated with mortality. Patients with group 3 PH are older, have more severe baseline presentation and lower survival rates than PAH patients in univariate analysis, that seemed to be related to older age.

Despite recent advances in lung transplantation, Aspergillus infections are still a major concern in lung transplant (LT) recipients. The genus Aspergillus may manifest as colonization or infection forms. The latter being associated with significant mortality and morbidity. Colonization, defined as bronchoalveolar lavage or bronchial aspirate, or positive bronchoalveolar lavage galactomannan test, or at least two positive sputum cultures, or tracheal aspirate for Aspergillus spp. in asymptomatic patients with normal-appearing respiratory mucosa or absence of endobronchial lesions, usually precedes infectious forms and complicates transplantation course. While several antifungal prophylactic strategies have been used to limit this deadly disease that mess with this complex endeavor, evidence from retrospective or prospective studies is lacking. In a recent international survey, only 50% of lung transplants' centers use antifungal prophylaxis, yet, substantial differences regarding the duration and the modality of antifungal prophylaxis exist among centers and, hence, increase the unmet need for clear recommendations. Since airways' colonization usually precedes Aspergillus infections and LT recipients are most vulnerable, in particular, during the early post-operative period due to anastomotic ischemia, Aspergillus' eradication seems to be a plausible solution to prevent or minimize Aspergillus colonization/infections post-transplantation. Consequently, all patients who underwent single or double lung transplantation in our center after 2014 received antifungal prophylaxis started on day 1 post-transplantation and carried on till healing of bronchial anastomosis. We report our 4-year experience with antifungal prophylaxis in LT recipients. A retrospective analysis was conducted and compared LT recipients who systematically received inhaled amphotericin B deoxycholate (6 mg twice daily) and 200 mg twice daily oral voriconazole (2014–2017) to those who did not (2008–2013). Only non-cystic fibrosis LT recipients were included in the analysis given that cystic fibrosis patients usually harbor aspergillus species in their sino-nasal/respiratory tract, a fact that may underestimate such therapeutic strategy benefit and Aspergillus' eradication may not be achieved. The data showed in this analysis was traced till hospital discharge. A total of 142 non-cystic fibrosis patients had undergone single/double lung transplantation. Between 2014 and 2017, 59 LT recipients received antifungal prophylaxis and Aspergillus spp. was found in 20 patients (incidence of 33%), while in the control group (n = 83) Aspergillus spp. was recovered from 42 patients (incidence of 50%). The difference between these 2 groups was statistically significant (P = 0.04) suggesting a potential beneficial effect of prompt post-operative antifungal prophylaxis in reducing Aspergillus colonization. Aspergillus fumigatus, A. niger, and A. flavus were most commonly found. Immediate post-operative antifungal prophylaxis using amphotericin B deoxycholate and oral voriconazole significantly reduced nosocomial Aspergillus colonization in non-cystic fibrosis LT recipients. A finding that might decrease subsequent Aspergillus related complications and reduce unnecessary morbidity. However, prospective controlled randomized trials with long-term follow-up are eagerly awaited.

Le pronostic des patients atteints d’hypertension artérielle pulmonaire (HTAP) hospitalisés en réanimation pour insuffisance cardiaque droite est caractérisé par une mortalité élevée. Notre objectif était d’analyser les caractéristiques et le pronostic des patients atteints d’HTAP du groupe 1 admis en réanimation quel que soit leur motif d’admission. Nous avons analysé de manière rétrospective les patients avec HTAP diagnostiquée entre 2003 et 2013 dans notre centre de compétences. Nous avons comparé les caractéristiques cliniques, biologiques, hémodynamiques et thérapeutiques entre les patients décédés en réanimation et les survivants. Au total, 28 patients ont été analysés, avec un âge médian de 54 ans. L’HTAP idiopathique était l’étiologie la plus représentée (28,6 %). Plus de la moitié des patients avaient une classe fonctionnelle NYHA III ou IV lors de la dernière évaluation à l’état stable avant admission en réanimation. Les 3 motifs d’entrée en réanimation les plus fréquents étaient la détresse respiratoire aiguë secondaire à une insuffisance cardiaque droite (35,6 %), l’arrêt cardio-respiratoire (17,9 %) et le choc septique (17,9 %). 16 patients sont décédés en réanimation soit un taux de mortalité de 57,1 %, au cours d’un séjour durant entre 0 et 21 jours. Des amines vasopressives ont été instaurées dans 75 % des cas, une ventilation invasive dans 50 % des cas, une épuration extra-rénale dans 28,6 % des cas, et un traitement par époprosténol a été introduit dans 14,3 % des cas. La comparaison entre les patients décédés et les survivants n’a pas retrouvé de différence significative au niveau de l’âge, de la classe NYHA, des comorbidités, du score IGS II, du motif d’admission ni au niveau des paramètres hémodynamiques obtenus lors du dernier cathétérisme cardiaque droit à l’état stable. Les seules différences statistiquement significatives retrouvées étaient un taux sanguin de transaminases et de troponine plus élevé chez les patients décédés, ainsi qu’un recours plus fréquent aux amines, à la dialyse et à la ventilation invasive. La mortalité des patients atteints d’HTAP admis en réanimation est élevée quel que soit le motif d’admission et ce malgré le recours aux techniques invasives de suppléance d’organe. En l’absence de facteur prédictif de décès, le transfert en réanimation des patients avec HTAP et en défaillance aiguë est justifié.

La fibroélastose pleuroparenchymateuse est une maladie fibreuse progressive du poumon et de la plèvre prédominant au niveau des territoires supérieurs. Aucun médicament n’a montré d’efficacité dans cette maladie. L’objective de cette étude est d’évaluer le bénéfice potentiel du nintédanib pour stabiliser la fonction pulmonaire. Nous avons examiné les données cliniques et les explorations fonctionnelles respiratoires des patients consécutifs admis dans le centre de référence des maladies pulmonaires rares, diagnostiqués avec fibroélastose pleuroparenchymateuse primaire ou secondaire, et qui ont reçu du nintédanib pendant au moins trois mois, à visée compassionnelle. Parmi 14 patients avec un diagnostic de fibroélastose pleuroparenchymateuse, 7 patients ont été traités par le nintédanib et ont été suivis pendant une durée médiane de 7,3 mois (de 6 à 24 mois). La fibroélastose était idiopathique dans 4 cas, et secondaire dans 3 cas. Une stabilisation de la capacité vitale forcée (CVF) semble avoir été obtenue après l’instauration du traitement dans la plupart des cas. La tolérance du nintédanib était globalement comparable à celle observée au cours de l’utilisation pour la fibrose pulmonaire idiopathique. Le nintédanib pourrait ralentir le déclin de la fonction pulmonaire chez les patients atteints de fibroélastose pleuroparenchymateuse idiopathique et secondaire. Ces observations nécessitent d’être confirmées par un essai contrôlé et un suivi prolongé.

Patient age at diagnosis of pulmonary hypertension is steadily increasing. The present study sought to analyse clinical characteristics, time to diagnosis and prognosis of pulmonary hypertension in elderly and very elderly patients.A study was conducted in a French regional referral centre for pulmonary hypertension. All consecutive patients diagnosed with pre-capillary pulmonary hypertension were included and categorised according to age: <65 years (“young”), 65–74 years (“elderly”) and ≥75 years (“very elderly”).Over a 4-year period, 248 patients were included: 101 (40.7%) were young, 82 (33.1%) were elderly and 65 (26.2%) were very elderly. The median age at diagnosis among the total population was 68 years. Compared with young patients, elderly and very elderly patients had a longer time to diagnosis (7±48, 9±21 and 16±32 months, respectively; p<0.001). Patients ≥75 years also more often had group 4 pulmonary hypertension. The median overall survival was 46±1.4 months, but was only 37±4.9 months in elderly patients and 28±4.7 months in very elderly patients. Survival from the first symptoms and survival adjusted to comorbidity was similar across age groups.Patient age should be taken into account when diagnosing pulmonary hypertension as it is associated with a specific clinical profile and a worse prognosis. The difference in prognosis is likely to be related to a delay in diagnosis and a greater number of comorbidities.

Introduction: Pleuroparenchymal fibroelastosis (PPFE) is a progressive fibrotic lung disease characterized by pleural thickening, pleural and parenchymal fibrosis predominately involving upper parts of the lungs. Survival is dismal and no drug has been shown to modify disease course. Aim and objectives Since parenchymal fibrosis is an important histological feature, we hypothesized that anti-fibrotic treatments might be effective in reducing disease progression. Here, we report our experience using nintedanib. Patients We reviewed patients diagnosed with PPFE and admitted to our center. Out of 11 patients, 5 received nintedanib (150 mg twice daily), were followed for at least 3 months. PPFE was idiopathic in 3 patients and secondary to chemotherapy in 2 patients. Patients were treated off-label at the discretion of the physician. Nintedanib was combined with 10 mg/day of prednisone inpatients #4 and #5. Results: In all 5 patients, including2 of them who had previously received pirfenidone, FVC declined steadily before initiation of nintedanib. Treatment with nintedanib was followed by apparent stabilization of FVC (Fig.1) with a median follow-up of 10 months (range, 3-13 months). Tolerability of nintedanib was generally good. Conclusion: Nintedanib may reduce FVC decline in patients with idiopathic and secondary PPFE.

L’âge lors du diagnostic d’hypertension pulmonaire (HTP) est en augmentation. Les objectifs de cette étude étaient d’évaluer les particularités cliniques et hémodynamiques, et le pronostic des patients âgés (65–74 ans) et très âgés (≥ 75 ans). Nous avons conduit une étude rétrospective monocentrique dans un centre de compétences régional de l’HTP. Tous les patients consécutifs diagnostiqués pour une HTP précapillaire ont été inclus dans l’analyse. Entre le1er novembre 2010 et le 31 octobre 2014, une HTP précapillaire a été diagnostiquée chez 248 patients, d’âge médian de 68 ± 14 ans. Au diagnostic, 82 patients (33,1 %) étaient âgés de 65 à 74 ans, et 65 (26,2 %) de 75 ans ou plus. L’étiologie variait en fonction de l’âge (p < 0,001) : l’HTP du groupe 1 était prédominante chez les patients jeunes (55,4 %) ; 40 % des patients âgés et très âgés avaient une HTP du groupe 3. Parmi les patients de 75 ans ou plus, 24,6 % avaient une HTP du groupe 4. Le délai de diagnostic augmentait avec l’âge (p = 0,001). La distance parcourue au test de marche de 6 minutes diminuait significativement avec l’âge (p < 0,001). Au diagnostic, 62 % des patients étaient en classe III/IV NYHA, quel que soit l’âge (p = 0,205). Les pressions artérielles pulmonaires étaient inférieures chez les patients de 65 ans et plus ; l’index cardiaque médian était diminué chez les patients de plus de 75 ans uniquement (tous p < 0,05). La médiane de survie était de 46 ± 1,4 mois dans la cohorte globale et de 28 ± 4,7 mois chez les ≥ 75 ans (p < 0,001). La survie à partir des premiers symptômes (p = 0,237) et la survie ajustée sur le score de comorbidités de Charlson (p = 0,343) ne différaient pas selon les classes d’âge. L’âge lors du diagnostic d’HTP précapillaire est corrélé à des particularités cliniques et hémodynamiques. Le retard de diagnostic et les comorbidités des patients les plus âgés participent au moins bon pronostic.