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International audience; BackgroundThe present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France.MethodsPractical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases.ResultsLymphangioleiomyomatosis is a rare lung disease characterised by a proliferation of smooth muscle cells that leads to the formation of multiple lung cysts. It occurs sporadically or as part of a genetic disease called tuberous sclerosis complex (TSC). The document addresses multiple aspects of the disease, to guide the clinicians regarding when to suspect a diagnosis of lymphangioleiomyomatosis, what to do in case of recurrent pneumothorax or angiomyolipomas, what investigations are needed to make the diagnosis of lymphangioleiomyomatosis, what the diagnostic criteria are for lymphangioleiomyomatosis, what the principles of management are, and how follow-up can be organised. Recommendations are made regarding the use of pharmaceutical specialties and treatment other than medications.ConclusionThese recommendations are intended to guide the diagnosis and practical management of pulmonary lymphangioleiomyomatosis.

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.

Background: There is a paucity of data on the prevalence of smoking among physicians. As physicians are on the front line caring for patients’ health, a detailed analysis of their smoking habits and its impact on counseling their patients to quit is warranted. So far, no study in Lebanon has addressed the real prevalence of physicians smoking and its impact on promoting cessation. This study aims to fill this purpose. Methods: A cross-sectional study conducted using a web-based self-administered questionnaire was e-mailed to 4037 Lebanese physicians between November 2015 and February 2016. The questionnaire included 11 questions about basic sociodemographic information (age, gender, work province, specialty), smoking habits and attitude towards smoking cessation. SPSS was used for analysis. The main outcome was to calculate the percentage of physicians that smoke, its relationship to gender and age, and its impact on counseling their patients about smoking cessation. Results: 529 responders were analyzed (13.1% response rate). A high rate of physician ever-smokers was noted at 37% (n=195) whereas the prevalence of current smokers was 13% (n=70).. In addition, there was a difference in the gender of ever-smoking physicians where 47% of males were smokers compared to 20% of females only. Regarding the prevalence of smoking within different specialties, it was noted that 35% (n=74) of internal medicine physicians were ever-smokers compared to approximately 50% (n=51/102) of surgeons. There was a statistically significant difference between former-smokers and current-smokers in regards to how frequently they urge their patients to quit smoking. Conclusion: The high prevalence of smoking among Lebanese physicians is depicted and its negative impact on counseling patients to quit is a serious consequence. This is a major drawback in the fight against tobacco and further awareness may be needed among our future doctors to increase smoking cessation counseling and decrease the burden of smoking in Lebanon and worldwide.

BACKGROUND While a number of individual patient characteristics are associated with survival in idiopathic pulmonary fibrosis (IPF), their incorporation into combined indexes, such as the GAP index, has been shown to increase the predictive capacity. It is unknown whether the predictive capacity of GAP-derived indexes that also include anthropometric and exercise parameters is superior to the original instrument. METHODS We tested the four-year survival predictive capacity of a modified, adimensional and multiplicative GAP index (IC4) that included percent forced vital capacity (FVC%), diffusing capacity of the lung for carbon monoxide (DLCO%), body mass index (BMI), and six-minute walk distance (6MWD) in 90 IPF patients recruited from two centres in France and Italy. RESULTS In ROC comparisons, the AUC of the IC4 (0.859, 95% CI 0.770-0.924 p

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International audience

Background There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. An algorithm for extracting claims data was developed to indirectly identify and describe patients with PF-ILD in the French national administrative healthcare database. Methods The French healthcare database, the Système National des Données de Santé (SNDS), includes data related to ambulatory care, hospitalisations and death for 98.8% of the population. In this study, algorithms based on age, diagnosis and healthcare consumption were created to identify adult patients with PF-ILD other than idiopathic pulmonary fibrosis between 2010 and 2017. Incidence, prevalence, survival estimates, clinical features and healthcare resource usage and costs were described among patients with PF-ILD. Results We identified a total of 14,413 patients with PF-ILD. Almost half of them (48.1%) were female and the mean (± standard deviation) age was 68.4 (± 15.0) years. Between 2010 and 2017, the estimated incidence of PF-ILD ranged from 4.0 to 4.7/100,000 person-years and the estimated prevalence from 6.6 to 19.4/100,000 persons. The main diagnostic categories represented were exposure-related ILD other than hypersensitivity pneumonitis (n = 3486; 24.2%), idiopathic interstitial pneumonia (n = 3113; 21.6%) and rheumatoid arthritis-associated ILD (n = 2521; 17.5%). Median overall survival using Kaplan–Meier estimation was 3.7 years from the start of progression. During the study, 95.2% of patients had ≥ 1 hospitalisation for respiratory care and 34.3% were hospitalised in an intensive care unit. The median (interquartile range) total specific cost per patient during the follow-up period was €25,613 (10,622–54,287) and the median annual cost per patient was €18,362 (6856–52,026), of which €11,784 (3003–42,097) was related to hospitalisations. Limitations included the retrospective design and identification of cases through an algorithm in the absence of chest high-resolution computed tomography scans and pulmonary function tests. Conclusions This large, real-world, longitudinal study provides important insights into the characteristics, epidemiology and healthcare resource utilisation and costs associated with PF-ILD in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and healthcare services. Trial registration ClinicalTrials.gov, NCT03858842. ISRCTN, ISRCTN12345678. Registered 3 January 2019—Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03858842

Birt-Hogg-Dubé (BHD) syndrome is a rare autosomal-dominant disorder, affecting multiple organs, mostly the skin, lungs, and kidneys. The prevalence of BHD syndrome is difficult to define given the rarity of the disease. Patients present most often with primary spontaneous pneumothorax. Renal tumors are a characteristic finding in BHD, and are often bilateral and multifocal and of the chromophobe and oncocytoma variant. Very scarce reports have highlighted the presence of simple renal cysts, as the only phenotypical renal manifestation, in BHD patients. Herein, we highlight two novel cases of bilateral multiple renal and pelvic cysts, in two females with genetically proven BHD syndrome, doubting a potential association with BHD syndrome.

International audience; Introduction Chronic pulmonary eosinophilia is a rare condition, usually highly responsive to systemic glucocorticoids, yet relapses are frequent and require long-term treatment associated with significant morbidity. Areas covered We review the main conditions causing chronic pulmonary eosinophilia and glucocorticoid-sparing agents in this setting. Expert opinion An individually tailored etiologic assessment is mandatory in all patients presenting with chronic pulmonary eosinophilia. Reducing the cumulative exposure to glucocorticoids by treating flares with short courses of systemic glucocorticoids is both safe and feasible in idiopathic chronic eosinophilic pneumonia. In frequently relapsing patients and in those requiring high doses of glucocorticoids, alternatives to glucocorticoids should be considered. Mepolizumab has been approved in several countries for the treatment of both hypereosinophilic syndrome and eosinophilic granulomatosis with polyangiitis. Given their ability to induce rapid and sustained depletion in blood and tissue eosinophils (e.g. benralizumab and lirentelimab) and/or to curb Type 2-mediated inflammation (e.g. dupilumab), other biologics hold promise in these settings. Likewise, anti-IgE targeted therapies (i.e omalizumab) should be considered in the glucocorticoid-sparing therapeutic armamentarium of allergic or hypersensitivity-related diseases, including allergic bronchopulmonary aspergillosis. Other drugs (fevipiprant, tezepelumab) are at an early phase of development. Cost-effectiveness studies are lacking. Whether glucocorticoid-free treatment regimens are achievable in these conditions is unknown.

Variable patterns of disease progression are typically observed in patients with idiopathic pulmonary fibrosis (IPF). We sought to determine the prognostic capacity of blood cell count indexes, derived from routine complete blood cell (CBC) count, in a cohort of IPF patients. The neutrophil-to-lymphocyte ratio (NLR), derived neutrophil-to-lymphocyte ratio (dNLR), monocyte-to-lymphocyte ratio (MLR), platelet-to-lymphocyte ratio (PLR), systemic inflammation index (SII), systemic inflammation response index (SIRI), and aggregate index of systemic inflammation (AISI) were calculated at baseline in a consecutive series of 82 IPF patients followed for four years. After adjusting for age, gender, body mass index, smoking status, and disease stage, only the AISI was significantly associated with mortality (HR 1.0013, 95% CI 1.0003–1.0023, p = 0.015). Patients with AISI &lt, 434 and ≥434 had a median survival from the diagnosis of 35.3 ± 15.2 and 26.6 ± 16.3 months (p = 0.015), and a four-year survival rate of 54% and 34%, respectively. The AISI, easily derivable from routine laboratory tests, is independently associated with mortality in patients with IPF. Prospective studies in larger cohorts are required to confirm this association.

Tuberous sclerosis complex (TSC) is a rare multisystem genetic disorder affecting almost all organs with no sex predominance. TSC has an autosomal-dominant inheritance and is caused by a heterozygous mutation in either theTSC1orTSC2gene leading to hyperactivation of the mammalian target of rapamycin (mTOR). TSC is associated with several pulmonary manifestations including lymphangioleiomyomatosis (LAM), multifocal micronodular pneumocyte hyperplasia (MMPH) and chylous effusions. LAM is a multisystem disorder characterised by cystic destruction of lung parenchyma, and may occur in either the setting of TSC (TSC-LAM) or sporadically (S-LAM). LAM occurs in 30–40% of adult females with TSC at childbearing age and is considered a nonmalignant metastatic neoplasm of unknown origin. TSC-LAM is generally milder and, unlike S-LAM, may occur in males. It manifests as multiple, bilateral, diffuse and thin-walled cysts with normal intervening lung parenchyma on chest computed tomography. LAM is complicated by spontaneous pneumothoraces in up to 70% of patients, with a high recurrence rate. mTOR inhibitors are the treatment of choice for LAM with moderately impaired lung function or chylous effusion. MMPH, manifesting as multiple solid and ground-glass nodules on high-resolution computed tomography, is usually harmless with no need for treatment.

Objectives To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF. Methods We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann–Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated. Results In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p vol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p vol loss of 7.9% was associated with major adverse events (log-rank, p p Conclusions Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD. Key Points • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p 0.0001).

International audience

Prostate cancer is the most commonly diagnosed malignancy and the second most common cause of death in men after lung cancer. Isolated pulmonary metastasis from prostate cancer, without bone or lymph node involvement, is rare and accounts for less than 1% of cases. The diagnosis of solitary lung metastasis is even more challenging in patients with concomitant pulmonary disease and often mandates tissue biopsy from the lung nodule. We herein present a case of an elderly man with idiopathic pulmonary fibrosis who presented with a solitary lung nodule three years after a laparoscopic radical prostatectomy for localized prostate cancer. Initially thought as a primary lung lesion secondary to his pulmonary fibrosis, further workup and ultimately a lung segmentectomy proved a metastatic prostatic adenocarcinoma. The serum prostatic specific antigen dropped to nadir following resection, and he remained stable six months later.

Methotrexate can induce subacute hypersensitivity pneumonitis, a potentially lethal condition that should lead to drug discontinuation; however, its use does not seem to be associated with increased risk of chronic fibrosing ILD in rheumatoid arthritishttps://bit.ly/3sCyjji

Background: Video-assisted surgical lung biopsy (SLB) is performed in 10–30% of cases to establish the diagnosis of idiopathic pulmonary fibrosis (IPF). Objectives: The aim of the study was to analyze the impact of SLB on lung function in patients eventually diagnosed with IPF. Methods: This is an observational, retrospective, monocentric study of all consecutive patients eventually diagnosed with IPF in multidisciplinary discussion who underwent SLB over 10 years in a specialized center. The primary end point was the variation in forced vital capacity (FVC) before and after the SLB. The secondary end points were the variations in forced expiratory volume in one second (FEV1), total lung capacity (TLC), carbon monoxide diffusion capacity (DLCO), and morbidity and mortality associated with the SLB. Results: In 118 patients who underwent SLB and were diagnosed with IPF, a relative decrease in FVC of 4.8% (p < 0.001) was found between measurements performed before and after the procedure. The mean FVC decrease was 156 ± 386 mL in an average period of 185 days, representing an annualized decline of 363 ± 764 mL/year. A significant decrease was also observed after SLB in FEV1, TLC, and DLCO. Complications within 30 days of SLB occurred in 14.4% of patients. Two patients (1.7%) died within 30 days, where one of them had poor lung function. Survival at 1 year was significantly poorer in patients with FVC Conclusion: In this uncontrolled study in patients ultimately diagnosed with IPF, SLB was followed by a significant decline in FVC, which appears to be numerically greater than the average decline in the absence of treatment in the literature. Summary at a Glance: This study evaluated the change in lung function in 118 consecutive patients diagnosed with idiopathic pulmonary fibrosis by surgical lung biopsy. Forced vital capacity decreased by 156 ± 386 mL in a mean of 185 days between the last measurement before and first measurement after biopsy, representing an annualized decline of 363 ± 764 mL/year.

Some patients with interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis exhibit a progressive clinical phenotype. These chronic progressive fibrosing ILDs have a variety of underlying diseases, and their prevalence is currently unknown. Here we carry out the first systematic review of literature on the prevalence of fibrosing ILDs and progressive fibrosing ILDs using data from physician surveys to estimate frequency of progression among different ILDs. We searched MEDLINE and Embase for studies assessing prevalence of ILD, individual ILDs associated with fibrosis and progressive fibrosing ILDs. These were combined with data from previously published physician surveys to obtain prevalence estimates of each chronic fibrosing ILD with a progressive phenotype and of progressive fibrosing ILDs overall. We identified 16 publications, including five reporting overall ILD prevalence, estimated at 6.3–76.0 per 100,000 people in Europe (four studies) and 74.3 per 100,000 in the USA (one study). In total, 13–40% of ILDs were estimated to develop a progressive fibrosing phenotype, with overall prevalence estimates for progressive fibrosing ILDs of 2.2–20.0 per 100,000 in Europe and 28.0 per 100,000 in the USA. Prevalence estimates for individual progressive fibrosing ILDs varied up to 16.7 per 100,000 people. These conditions represent a sizeable fraction of chronic respiratory disorders and have a high unmet need. Electronic supplementary material The online version of this article (10.1007/s12325-020-01578-6) contains supplementary material, which is available to authorized users.

BK virus (BKV) pneumonia is a rare entity especially seen in immunosuppressed patients, for which cidofovir is the used treatment option. We describe a case of a young female patient who presented for altered kidney function six months following kidney transplantation for focal segmental glomerulosclerosis and was found to have BKV nephritis. Her in-hospital stay was complicated by BKV pneumonia requiring mechanical ventilation, in addition to CMV colitis. She was treated with leflunomide/ciprofloxacin and ganciclovir for her pneumonia and colitis, respectively. The patient improved clinically except that her kidney function deteriorated. Leflunomide/ciprofloxacin combination may constitute an effective and safe alternative to cidofovir for the treatment of BKV pneumonia, in particular when cidofovir is not available. J Microbiol Infect Dis 2021; 11(1):36-41.

Aims: ILDs are a heterogeneous group of disorders with distinct clinical, radiological and prognostic characteristics, which encompass a wide range of conditions. In some patients with fibrosing ILDs, a progressive phenotype similar to that observed in IPF may develop during the course of the disease. The collective estimated prevalence of PF-ILDs is 0.22, 0.59, and 2.0 per 10.000 persons in Belgium, Greece, and France respectively. The PROGRESS study aimed at assessing the epidemiology of non-IPF PF-ILD patients and associated health consumptions and outcomes in a real life nationwide setting in France. Methods: A longitudinal retrospective cohort study was conducted using the French administrative healthcare database (SNDS) between January 2010 and December 2017. Non-IPF PF-ILD patients were identified using an US algorithm based on ICD-10 codes and adapted to French specificities. Results: 17,560 non-IPF PF-ILD patients were identified. In 2017, the prevalence and incidence of non-IPF PF-ILD were respectively 17.9 per 100.000 and 3.0 per 100.000 per year. Sex ratio was close to 1 (51.2% of men) and mean age was 68.5 years (±15.2). Major underlying ILD diagnoses were non-autoimmune-related (50.0%), autoimmune-related (40.0%) and sarcoidosis (10.0%). The median overall survival of non-IPF PF-ILD patients was estimated at 4.1 years. The survival rate was 76.0%, 57.7%, 44.5%, and 33.7% at 1, 3, 5, and 8 years respectively. Conclusion: Based on an algorithm, non-IPF PF-ILD is rare and is associated with a poor prognosis comparable to that of IPF.

Aim: This retrospective, monocentric cohort study aimed at assessing clinical characteristics, lung function course and overall survival of patients with non-IPF PF-ILD in a real-world setting. Methods: All successive patients hospitalized in a French Rare Lung Disease Reference Centre between 2010 and 2017, aged ≥18 years, with at least 10% fibrosis extent at baseline high resolution computed tomography of chest (HRCT) were included. PF-ILD over a period of 2 years was defined as: relative decline in forced vital capacity (FVC) ≥ 10% with or without clinical deterioration (a); or a moderate relative decline between 5% and 10% associated with worsening respiratory symptoms or increased extent of fibrosis in HRCT (b); or increased extent of fibrosis on HRCT with worsening respiratory symptoms with or without FVC decline (c). Results: 165 patients (57% of women) were included with a mean age of 60 (±14 years). Most of patients (65%) were included with criterion (a), 24% with criterion (b) and 9% with criterion (c). Estimates of overall survival were 99%, 82%, and 66% at 1, 3 and 6 years, respectively. Mortality was significantly associated with FVC Conclusion: The natural history of non-IPF ILD in patients with PF phenotype follows a course characterized by worsening of respiratory symptoms, progressive lung function decline and early mortality despite appropriate management.

Aims: Interstitial lung disease (ILD) is frequent in patients with systemic sclerosis (SSc). It is characterized by progressive decline in lung function and is the leading cause of death in patients with SSc. In Europe, the prevalence and annual incidence of SSc-ILD have been estimated at 1.7-4.2 per 100,000 and 0.1-0.4 per 100,000 per year, respectively. This study aimed at assessing the epidemiology and mortality of patients with SSc-ILD in a real life nationwide setting in France. Methods: A longitudinal retrospective cohort study was conducted in the French administrative health care database. SSc-ILD patients were identified using an algorithm based on ICD-10 codes of SSc and of lung fibrosis between January 2010 and December 2017. Results: Among 9817 SSc patients identified, 3460 had associated ILD (35.0%). In 2017, the prevalence and incidence of SSc-ILD were respectively 4.5 per 100,000 (4.3-4.7) and 0.4 per 100,000 per year (0.3-0.5). Patients were predominantly females (75.6%) with a mean age of 60.7 years (±14.5). 73.6% and 20.8% of patients respectively had at least one claim of systemic corticosteroids and mycophenolate mofetil treatments during the study period. The overall survival rate was estimated to be 92.9%, 81.8%, 70.5%, and 65.1% 1, 3, 5, and 8 years, respectively. Median survival was better in women. Conclusion: This study demonstrated that SSc-ILD is a rare disease with significant mortality.

Background: Lung cancer is one of the most prevalent cancers worldwide. Chemotherapy regimens, targeted against lung cancer, are considered an effective treatment, albeit with multiple fatal side effects. An alternative strategy, nowadays, is using natural products. Medicinal plants have been used, in combination with chemotherapy, to ameliorate side effects. Aims: This study aims to investigate the antitumor effect of pomegranate juice (Punica granatum) on human lung adenocarcinoma basal epithelial cells (A549), to check the effect, when combined with low dose cisplatin (CDDP), at different doses. We also have evaluated the potential protective effect of pomegranate on normal peripheral blood mononuclear cells (PBMC). Methods: Phytochemical screening of the extract was done using standard classical tests. Total phenolic and sugar contents were determined using the Folin&ndash, Ciocalteu and anthrone reagents, respectively. The antioxidant activity of pomegranate was estimated by the 2,2-diphenyl-1-picrylhydrazyl (DPPH) method. The viability of A549 cells and PBMC was evaluated using the neutral red assay. Results: Our results demonstrated that Punica granatum or pomegranate juice (with different concentrations: 150, 300, 600 &micro, g/mL) contained high levels of flavonoids, alkaloids, tanins, lignins, terpenoids, and phenols. The DPPH method showed that pomegranate juice had a strong antioxidant scavenging activity. Neutral red showed that combining pomegranate juice with low dose CDDP (8 &micro, g/mL) decreased the cell viability of A549 cells, by 64%, compared to treatment with CDDP or pomegranate alone. When added to low dose CDDP, pomegranate increased the viability of normal PBMC cells by 46%. Conclusions: These results demonstrated that pomegranate could potentiate the anticancer effect of low dose CDDP on human lung adenocarcinoma cells (A549 cells) and could as well decrease its toxicity on PBMC.

Purpose of review To summarize the latest publications and provide a practical overview of treatment strategies for lung vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Recent findings In patients with severe ANCA-associated vasculitis, plasma exchange, as adjunctive therapy to standard treatment, is not associated with improved survival or reduced risk of end-stage kidney disease. A regimen with reduced dose of glucocorticoids is equally effective to induce remission as a standard regimen. In patients without organ or life-threatening disease, mycophenolate mofetil can be used in combination with oral glucocorticoid therapy to induce remission, however, with a higher risk of relapse than when using rituximab or cyclophosphamide. For maintenance of remission, a tailored regimen of rituximab infusion was equivalent to a fixed regimen, with fewer perfusions. Belimumab, a human IgG1(Equation is included in full-text article.)monoclonal antibody against B-lymphocyte stimulator, did not decrease the relapse rate when added to azathioprine and glucocorticoids. Avacopan, a complement C5a receptor inhibitor, was effective in replacing high-dose glucocorticoids in achieving complete remission of vasculitis. Summary Significant advances have been made in the treatment strategy to both induce remission and maintain remission in patients with ANCA-associated vasculitis. The choice should take into consideration efficacy, cost-effectiveness, safety profile, ease of use, and possibility of individual tailoring of treatment.

Epub ahead of print; International audience

Resume Les pneumopathies interstitielles diffuses (PID) sont souvent rencontrees au cours des connectivites. Certains patients ayant une pneumopathie interstitielle presentent des manifestations auto-immunes insuffisantes pour retenir le diagnostic de connectivite. La prise en charge de ce groupe de patients par le pneumologue est rendue difficile, car la frontiere entre les PID idiopathiques et les connectivites est parfois floue. Une prise en charge multidisciplinaire avec un rhumatologue experimente dans les maladies auto-immunes est conseillee en cas de suspicion de connectivite. Plusieurs classifications ont ete proposees dans le but d’uniformiser ce groupe de patients, mais aucune n’avait fait l’objet d’un consensus. Recemment, un groupe de travail issu de l’American Thoracic Society et de l’European Respirtory Society a propose une nouvelle nomenclature et des criteres consensuels definissant les PID avec manifestations auto-immunes (« interstitial pneumonia with auto-immune features » ou IPAF) dans le but d’uniformiser et de mieux caracteriser ce groupe de patients. Dans cette monographie, nous etudierons ce nouveau concept d’IPAF, apres avoir detaille les concepts anterieurs. Nous decrirons ensuite les recentes cohortes issues de cette nouvelle classification et les perspectives futures qui restent a approfondir.

Introduction La sclerodermie systemique (ScS) est une maladie auto-immune rare, heterogene et chronique. Les pneumopathies interstitielles diffuses (PID) caracterisees par un declin progressif de la fonction respiratoire constituent une complication frequente de la ScS et sa principale cause de deces. En Europe, la prevalence de la PID-ScS a ete estimee a 1,7–4,2/100 000 individus et l’incidence annuelle a 0,1–0,4/100 000 individus. En France, l’epidemiologie de cette maladie est encore meconnue. Ainsi, les objectifs de cette etude etaient d’estimer la prevalence et l’incidence de la PID-ScS en France et de decrire les caracteristiques et la survie des patients. Methodes Une cohorte retrospective longitudinale a ete constituee a partir des donnees du Systeme national des donnees de sante entre 2010 et 2017. Les patients ont ete identifies comme etant atteints d’une PID-ScS grâce a un algorithme base sur l’utilisation de codes CIM-10. Ainsi, tous les patients de plus de 20 ans, affilies au regime general d’Assurance Maladie depuis plus de deux ans, et avec: – au moins une hospitalisation ou une affection de longue duree relative a une ScS; – au moins une hospitalisation avec un code de fibrose pulmonaire ont ete inclus. Resultats Parmi les patients identifies avec une ScS, 34 % presentaient une PID, soit 3333 patients. Les trois quarts etaient des femmes (75,6 %). La moyenne (ecart-type) d’âge etait de 60,6 (± 14,4) ans. Les principales comorbidites etaient l’hypertension (66,8 %) et le reflux gastro-œsophagien (65,8%). L’incidence globale estimee etait de 0,98–0,76/100 000 individus et la prevalence de 3,42–5,52/100 000 individus sur l’ensemble de la periode. Les taux de prevalence et d’incidence etaient trois fois plus eleves chez les femmes que chez les hommes et augmentaient avec l’âge. Les estimations de la survie globale etaient de 93 % a 1 an, 82% a 3 ans, 71 % a 5 ans et 55% a 8 ans. Les facteurs associes a la mortalite etaient le sexe masculin, la presence d’une hypertension pulmonaire, la presence d’un cancer du poumon et les classes d’âges les plus elevees. Conclusion Cette etude presente pour la premiere fois au niveau national l’epidemiologie de la PID-ScS. Notamment, elle a permis de montrer que la PID-ScS presente un taux de mortalite eleve, en particulier chez les patients a risque.

Introduction Les pneumopathies interstitielles diffuses (PID) regroupent un ensemble heterogene de maladies caracterisees par un processus inflammatoire diffus. Chez certains patients atteints de PID, un phenotype fibrosant progressif (FP) se developpe, comparable a celui observe dans la fibrose pulmonaire idiopathique (FPI). L’objectif de l’etude etait de fournir les caracteristiques des patients et les estimations d’incidence, de prevalence et de mortalite de la PID-FP (hors fibrose pulmonaire idiopathique) a l’echelle nationale en vie reelle. Methodes Une cohorte retrospective longitudinale de patients atteints de PID-FP a ete constituee a partir des donnees du Systeme National des Donnees de Sante (SNDS). Les patients PID-FP ont ete selectionnes selon un algorithme d’identification base sur l’utilisation de codes CIM-10 en trois etapes successives: identification des PID, exclusion des FPI, et selection des FP. Resultats 30 771 patients atteints de PID hors FPI ont ete identifies. Parmi eux, 14 413 patients (47 %) susceptibles de presenter une forme progressive ont ete inclus. Un peu plus de la moitie des patients etaient des hommes (52 %). L’âge moyen (ecart-type) etait de 68,4 ans (± 15,0). Les comorbidites les plus frequentes etaient l’hypertension (64 %) et le reflux gastro-œsophagien (55 %). La prevalence des patients avec un phenotype progressif variait de 6,6 a 19,4/100 000 individus selon l’annee et l’incidence annuelle de 3,1 a 4,7/100 000 individus. Les taux de prevalence et d’incidence etaient legerement plus faibles chez les femmes que chez les hommes, et augmentaient considerablement avec l’âge. La mediane de la survie globale a ete estimee a 3,7 ans [3,6–3,8]. Le taux de survie etait de 74 % a 1 an, 55 % a 3 ans, 42 % a 5 ans et 32 % a 8 ans. Les facteurs associes a la mortalite etaient le sexe masculin, l’âge et l’etiologie de la PID-FP. Conclusion Dans cette etude, 47 % des patients atteints de PID etaient susceptibles de presenter une forme progressive comparativement a environ 30 % dans la litterature. Cette difference pourrait s’expliquer par l’utilisation pour la selection des patients avec FP, d’un algorithme base sur l’utilisation de consommations de soin, sans possibilite de validation diagnostique des formes progressives. En conclusion, cette etude qui est la premiere a estimer l’epidemiologie des PID-FP en France grâce a l’utilisation du SNDS, suggere que les PID-FP representent au total un assez grand nombre de patients, et sont associees a un mauvais pronostic.