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Sarcoidosis is an inflammatory multisystemic disease of unknown etiology characterized by the formation of noncaseating epithelioid cell granulomas. Cardiac sarcoidosis might be life-threatening and its diagnosis and treatment remain a challenge nowadays. The aim of this review is to provide an updated overview of cardiac sarcoidosis and, through 10 practical clinical questions and real-life challenging case scenarios, summarize the main clinical presentation, diagnostic criteria, imaging findings, and contemporary treatment. Résumé: La sarcoïdose est une maladie inflammatoire multisystémique de cause inconnue, caractérisée par la formation de granulomes non caséeux composés de cellules épithélioïdes. La sarcoïdose cardiaque est une pathologie potentiellement mortelle qui demeure, à ce jour, difficile à diagnostiquer et à traiter. L'objectif de cet article est de présenter les données les plus à jour concernant la sarcoïdose cardiaque et de résumer, à l'aide de 10 questions cliniques et de cas réels, la présentation clinique, les critères diagnostiques, les trouvailles à l'imagerie et le traitement contemporain.

In a proportion of patients with hypersensitivity pneumonitis, the biological and environmental factors that sustain inflammation are ill defined, resulting in no effective treatment option. Bioaerosols found in occupational settings are complex and often include Toll-like receptor ligands, such as endotoxins. How Toll-like receptor ligands contribute to the persistence of hypersensitivity pneumonitis, however, remains poorly understood. In a previous study, we found that an S1P(1) (sphingosine-1-phosphate receptor 1) agonist prevented the reactivation of antigen-driven B-cell responses in the lung. Here, we assessed the impact of endotoxins on B-cell activation in preexisting hypersensitivity pneumonitis and the role of S1P(1) in this phenomenon. The impact of endotoxins on pre-established hypersensitivity pneumonitis was studied in vivo. S1P(1) levels were tracked on B cells in the course of the disease using S1P(1)-eGFP knockin mice, and the role of S1P(1) on B-cell functions was assessed using pharmacological tools. S1P(1) was found on B cells in experimental hypersensitivity pneumonitis. Endotoxin exposure enhanced neutrophil accumulation in the BAL of mice with experimental hypersensitivity pneumonitis. This was associated with enhanced CD69 cell-surface expression on lymphocytes in the BAL. In isolated B cells, endotoxins increased cell-surface levels of costimulatory molecules and CD69, which was prevented by an S1P(1) agonist. S1P(1) modulators also reduced TNF production by B cells and their capacity to trigger T-cell cooperation ex vivo. An S1P(1) ligand directly inhibited endotoxin-induced B-cell activation.

PURPOSE To assess the 1-min sit-to-stand test (1STS) test-retest reliability and construct validity and its associated cardiorespiratory response in comparison to the 6-min walk test (6MWT) and symptom-limited cycling cardiopulmonary exercise test (CPET) in people with interstitial lung disease (ILD). METHODS Fifteen participants with ILD performed two 1STS tests, a 6MWT and a CPET. The three tests were administered on three separate visits, and cardiorespiratory parameters were continuously recorded during the tests. RESULTS The number of repetitions during both 1STS tests was 22 ± 4 and 22 ± 4 (mean difference of 0.53 ± 2.00 repetitions, P = 0.32) with an intraclass correlation of 0.937 (95% confidence interval, 0.811-0.979]) and a minimal detectable change of 2.9 repetitions. The number of 1STS repetitions was highly correlated with the 6MWT distance (r = 0.823, P < 0.001) and with the peak cycling power output expressed in % predicted values (r = 0.706, P < 0.003). Oxygen consumption (V˙O2) peak during the 1STS reached 83% and 78% of V˙O2 peak during 6MWT and CPET, respectively. Peak 1STS HR, minute ventilation (V˙E,), V˙O2 values, as well as nadir SpO2 were achieved during the recovery phase of the test, whereas peak 6MWT and CPET HR, V˙E, V˙O2 and nadir SpO2 always occurred at the end of the test. The three tests elicited a similar fall in SpO2 ranging between 8% and 12%. Symptom scores after the 1STS were similar to those seen at the end of the 6MWT but lower than those of CPET. CONCLUSIONS The 1STS showed excellent test-retest reliability in patients with ILD in whom it elicited a substantial, but submaximal cardiorespiratory response. Our data also support the construct validity of the 1STS to assess functional exercise capacity in patients with ILD and to detect exercise-induced O2 desaturation.

Background Surgical lung biopsy (SLB) is considered in the investigation of interstitial lung diseases (ILDs) when a complete clinical evaluation and a multidisciplinary discussion (MDD) do not allow the clinician to make a confident diagnosis. Owing to the risk of the procedure, an appropriate assessment of the risk/benefit ratio prior to the intervention is recommended. We aimed to assess the postoperative outcomes and diagnostic yield of SLB for the investigation of ILD in a tertiary care institution. Methods We conducted a retrospective cohort study of consecutive subjects who underwent a SLB for the investigation of ILD in our center from 2009 to 2020. The postoperative mortality and complications rates as well as the diagnostic yield of the procedure were assessed. Results Of the 1,805 patients newly investigated for ILD in our center from 2009 to 2020, 71 (3.93%) underwent a SLB. At days 30 and 90, the mortality rates were 0 and 2.8%, whereas 4.3 and 7.6% patients experienced an acute ILD exacerbation, respectively. In addition, 4 (5.8%) patients experienced infectious complications and 5 (7.0%) presented prolonged air leaks (all within 30 days). A definite pathological diagnosis was made in 47 (66.2%) patients. Following postoperative MDD, a confident diagnosis was made in 61 patients (85.9%) and resulted in a change of therapy in 49 (69.0%) patients. Conclusion SLB for the diagnosis of unclassifiable ILDs is associated with low mortality but significant morbidity. However, it results in a confident diagnosis and a change in therapy in the majority of patients.

Introduction: This study assesses the effects of automated oxygen titration, alone or with high flows on dyspnea and exercise tolerance in patients with chronic lung disease and exercise-induced O2 desaturation. Methods: Patients with chronic lung diseases and exercise desaturation were involved in a 3 treatment arm cross-over study to perform a 3-min constant speed walk test (3min CSST) and an endurance shuttle walking test (ESWT) carried out with one of the 3 oxygen delivery systems: (1) O2 at fixed-flow of 2 L/min (2) or with an automated O2 titration system (FreeO2®) targeting 94% SpO2, and (3) FreeO2 in + high flow nasal cannula (FreeO2 + Airvo®). The main outcome was the dyspnea score (modified Borg scale) following 3min CSST. Secondary outcomes were endurance time and mean/nadir SpO2 during ESWT. Results: In this interim analysis, 14 patients (7 COPD and 4 ILD, 2 PH and 1 CF) were included. There was no difference in the dyspnea score. Endurance time was longer (p Conclusion: These preliminary results suggest that, despite no improvement in dyspnea, automatic O2 titration increases endurance time and SpO2 during ESWT compared to fixed-flow O2. Adding AirvoTM had no further benefits.

Context The associations of serum 25-hydroxyvitamin D [25(OH)D] with plasma lipids remain controversial in children. Objective To examine the associations and interactions of 25(OH)D and related gene variants with lipids in children. Design Cross-sectional. Setting Kuopio, Finland. Participants Population sample of 419 prepubertal white children aged 6 to 8 years. Main Outcome Measures 25(OH)D, total cholesterol, low-density lipoprotein (LDL) cholesterol, high-density lipoprotein (HDL) cholesterol, and triglycerides. Results Serum 25(OH)D was negatively associated with total cholesterol (β = -0.141, P = 0.004), LDL cholesterol (β = -0.112, P = 0.023), HDL cholesterol (β = -0.150, P = 0.002), and triglycerides (β = -0.104, P = 0.035) adjusted for age and sex. Associations of 25(OH)D with total cholesterol, LDL cholesterol, and HDL cholesterol remained after adjustment for adiposity, physical activity, sedentary behavior, diet, daylight time, and parental education. Children in the highest quartile of 25(OH)D had the lowest total cholesterol (P = 0.022) and LDL cholesterol (P = 0.026) adjusted for age and sex. Cytochrome P450 family 2 subfamily R member 1 (CYP2R1) rs12794714, CYP2R1 rs10741657, and vitamin D binding protein (DBP) rs2282679 were associated with 25(OH)D adjusted for age and sex. CYP2R1 rs12794714 was associated with total cholesterol and LDL cholesterol and C10orf88 rs6599638 with HDL cholesterol adjusted for age, sex, and 25(OH)D. The gene variants did not explain or modify the associations of 25(OH)D with lipids. Conclusions 25(OH)D was independently and inversely associated with total cholesterol, LDL cholesterol, and HDL cholesterol. CYP2R1 rs12794714, CYP2R1 rs10741657, and DBP rs2282679 were associated with 25(OH)D. CYP2R1 rs12794714 was associated with total cholesterol and LDL cholesterol and chromosome 10 open reading frame 88 (C10orf88) rs6599638 with HDL cholesterol independent of 25(OH)D. None of the gene variants modified the associations of 25(OH)D with lipids. Further studies are needed to detect the mechanisms for the associations of 25(OH)D with lipids.

Fibrosis complicates numerous pathologies including interstitial lung diseases. Sphingosine analogs such as FTY720 can alleviate lung injury-induced fibrosis in murine models. Contradictorily, FTY720 also promotes in vitro processes normally leading to fibrosis and high doses in vivo foster lung fibrosis by enhancing vascular leakage into the lung. The goal of this study was to determine the effect of low doses of FTY720 on lung fibrosis triggered by an acute injury in mice. We first defined the time-boundaries delimiting the inflammatory and remodelling phases of an injury elicited by bleomycin based on neutrophil counts, total lung capacity and lung stiffness. Thereafter, FTY720 (0.1 mg/kg) was delivered during either the inflammatory or the remodelling phases of bleomycin-induced injury. While FTY720 decreased fibrosis by 60% and lung stiffness by 28% when administered during the inflammatory phase, it increased fibrosis (2.1-fold) and lung stiffness (1.7-fold) when administered during the remodelling phase. FTY720-induced worsening of fibrosis was associated with an increased expression of connective tissue growth factor, but not with vascular leakage into the lung. Thus, the timing of FTY720 delivery following a bleomycin-induced lung injury determines pro-vs anti-fibrotic outcomes.

Pulmonary rehabilitation improves dyspnea and exercise capacity in idiopathic pulmonary fibrosis (IPF); however, it is unknown whether breathing high amounts of oxygen during exercise training leads to further benefits.Herein, we describe the design of the High Oxygen Delivery to Preserve Exercise Capacity in IPF Patients Treated with Nintedanib study (the HOPE-IPF study). The primary objective of this study is to determine the physiological and perceptual impact of breathing high levels of oxygen during exercise training in patients with IPF who are receiving antifibrotic therapy.HOPE-IPF is a two-arm double-blind multicenter randomized placebo-controlled trial of 88 patients with IPF treated with nintedanib. Patients will undergo 8 weeks of three times weekly aerobic cycle exercise training, breathing a hyperoxic gas mixture with a constant fraction of 60% inhaled oxygen, or breathing up to 40% oxygen as required to maintain an oxygen saturation level of at least 88%.End points will be assessed at baseline, postintervention (Week 8), and follow-up (Week 26). The primary analysis will compare the between-group baseline with post-training change in endurance time during constant work rate cycle exercise tests. Additional analyses will evaluate the impact of training with high oxygen delivery on 6-minute walk distance, dyspnea, physical activity, and quality of life.The HOPE-IPF study will lead to a comprehensive understanding of IPF exercise physiology, with the potential to change clinical practice by indicating the need for increased delivery of supplemental oxygen during pulmonary rehabilitation in patients with IPF. Clinical trial registered with www.clinicaltrials.gov (NCT02551068).

Background Biliopancreatic diversion with duodenal switch (BPD-DS) with a 100-cm common channel has been our treatment of choice for morbid obesity since the early 1990s. This procedure offers excellent long-term weight loss but can be associated with significant side effects. Objectives To assess the effect on clinical and nutritional parameters of increasing the common channel to 200 cm. Settings University-affiliated tertiary care center. Methods Patients who underwent a BPD-DS with a 200-cm common channel (study group, n=36) were matched 1:1 for age, sex, body mass index (BMI), and main co-morbidities with patients who underwent a BPD-DS with a 100-cm common channel (control group). The strict alimentary limb was 150 cm in both groups. Results The mean age was 55±9 versus 53±7 years ( P = .3), with 50% women and a BMI of 49±8 kg/m 2 versus 50±6 kg/m 2 ( P = .9). Follow-up rate was 97%, with a minimum follow-up of 3 years. There were no significant differences in the remission rate of major co-morbidities between the 2 groups. At 3 years, the excess weight loss was 61±22% versus 68±18% ( P = .18) and the total weight loss was 33±11% versus 38±9% ( P = .055) in the study group versus control group, respectively. The study group had a lower incidence of severe protein deficiency (11% versus 19%, P = .3) and hyperparathyroidism (17.1% versus 35.3%, P = .17); required a lower amount of vitamins A and D ( P P = .03). Conclusion In this population, BPD-DS with a 200-cm common channel offered similar remission rate of co-morbidities compared with standard BPD-DS. It was associated with similar weight loss at nadir, followed by a more significant weight regain. It might yield a lower rate of nutritional complications. Long-term randomized data are needed to detect other potential advantages.

Idiopathic pulmonary fibrosis (IPF) is a rare disease, with estimates of prevalence varying considerably across countries due to paucity in data collection. The aim of this study was to investigate the prevalence and incidence of IPF in Canada using administrative data requiring minimal extrapolation.We used mandatory national administrative data from 2007–2011 to identify IPF cases of all ages with an International Classification of Diseases (Version 10, Canadian) diagnosis code of J84.1. We used a broad definition that excluded cases with subsequent diagnosis of other interstitial lung diseases, and a narrow definition that required further diagnostic testing prior to IPF diagnosis. We explored survival and quality of life.For all ages, the broad prevalence of IPF was 41.8 per 100 000 (14 259 cases) and was higher for men. The incidence rate was 18.7 per 100 000 (6390 cases) and was higher for men. The narrow prevalence was 20.0 per 100 000 (6822 cases) and incidence was 9.0 per 100 000 (3057 cases). The 4-year risk of death was 41.0% and the quality of life with IPF after 2 years was lower than for Global Initiative for Chronic Obstructive Lung Disease stage IV chronic obstructive pulmonary disease.Using comprehensive national data, the prevalence of IPF in Canada was higher than other national estimates, suggesting that either IPF may be more common in Canada or that data capture may have been previously limited.

Jean-Eric Tarride,1,2 Robert B Hopkins,1,2 Natasha Burke,1,2 Jason R Guertin,3,4 Daria O’Reilly,1,2 Charlene D Fell,5 Genevieve Dion,6 Martin Kolb7 1Department of Health Research Methods, Evidence and Impact, Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada; 2Programs for Assessment of Technology in Health (PATH), The Research Institute of St. Joe’s Hamilton, St. Joseph’s Healthcare Hamilton, Hamilton, ON, Canada; 3Department of Social and Preventive Medicine, Laval University, Quebec City, QC, Canada; 4Centre de recherche du CHU de Québec – Université Laval, Axe Santé des Populations et Pratiques Optimales en Santé, Hôpital du St-Sacrement, Quebec City, QC, Canada; 5Division of Respirology, Department of Medicine, University of Calgary, Calgary, AB, Canada; 6Quebec Heart and Lung Institute, Laval University, Quebec City, QC, Canada; 7Division of Respirology, Department of Medicine, Faculty of Health Sciences, McMaster University, Hamilton, ON, Canada Background: Idiopathic pulmonary fibrosis (IPF), although rare, is a severe and costly disease.Objective: To estimate the clinical and economic burden of IPF over multiple years before and after diagnosis using comprehensive administrative databases for the province of Quebec, Canada.Methods: Several administrative databases from Quebec, providing information on hospital care, community care, and pharmaceuticals, were linked over a 5-year period ending March 31, 2011, which was before approval of antifibrotic drugs in Canada. Prevalent and incident IPF cases were defined using International Classification Disease-10-CA codes and International Classification Disease-9-CM codes. We used a broad definition that excluded cases with subsequent diagnosis of other interstitial lung diseases and a narrow definition that required further diagnostic testing to confirm IPF diagnosis. Incident cases had an IPF code in a particular year without any IPF code in the 2 previous years. Health care resource utilization before and after the index diagnosis date was determined and costs calculated. Costs were expressed in 2016 Canadian dollars.Results: Over 5-years, 10,579 (mean age: 76.4; 58% male) satisfied the broad definition of IPF and 8,683 (mean age: 74.5; 57% male) satisfied the narrow definition (82% of broad). Incidences of IPF overall were 25.8 and 21.7/100,000 population for broad and narrow definitions, respectively. Three-year survival was 40% and 37% in broad and narrow cohorts, respectively. For both cohorts, health care resource utilization and costs increased several years before diagnosis ($2,721 and $7,049/patient 5 years and 2 years prior to diagnosis using a broad definition, respectively) and remained elevated for multiple years post diagnosis ($12,978 and $8,267 at 2 and 3 years postdiagnosis).Conclusion: Health care resource utilization and costs of IPF increase many years prior to diagnosis. Incorporating multiyear annual costs before and after diagnosis results in a higher estimate of the economic burden of IPF than previous studies using a 1-year time frame. Keywords: cost of illness, health care utilization, incidence, idiopathic pulmonary fibrosis, Canada

Chronic lung consolidation has a limited number of differential diagnoses requiring distinct managements. The aim of the study was to investigate how logical analysis of data (LAD) can support their diagnosis at HRCT (high-resolution computed tomography).One hundred twenty-four patients were retrospectively included and classified into 8 diagnosis categories: sarcoidosis (n=35), connective tissue disease (n=21), adenocarcinoma (n=17), lymphoma (n=13), cryptogenic organizing pneumonia (n=11), drug-induced lung disease (n=9), chronic eosinophilic pneumonia (n =7) and miscellaneous (n=11). First, we investigated the patterns and models (association of patterns characterizing a disease) built-up by the LAD from combinations of HRCT attributes (n=51). Second, data were recomputed by adding simple clinical attributes (n=14) to the analysis. Third, cluster analysis was performed to explain LAD failures.HRCT models reached a sensitivity80% and a specificity90% for adenocarcinoma and chronic eosinophilic pneumonia. The same thresholds were obtained for sarcoidosis, connective tissue disease, and drug-induced lung diseases when clinical attributes were added to HRCT. LAD failed to provide a satisfactory model for lymphoma and cryptogenic organizing pneumonia, with overlap between both diseases shown on cluster analysis.LAD provides relevant models that can be used as a diagnosis support for the radiologist. It highlights the need to add clinical data in the analysis due to frequent overlap between diseases at HRCT.

Studies have shown that symmetric stimuli are recognized better than asymmetric stimuli but evidence suggests that this advantage may result from a familiarity bias induced by symmetry. We used a classic episodic memory paradigm to test this bias and see if it truly accounts for the symmetry advantage. Subjects first encoded symmetric and asymmetric figures. During a subsequent recognition phase, they discriminated the encoded (old) figures from new intermixed figures. The recognition rate of old figures was higher with symmetric figures than asymmetric figures. However, the tendency to falsely recognize new figures was also higher when they were symmetric, meaning that the higher recognition rate for symmetric figures was artificially inflated by a response bias. Three other experiments further tested this finding and examined the influence of some variables (rotation in virtual 3D space, stimulus meaningfulness, and redundancy of information) on the bias. A fifth experiment with photo stimuli confirmed that the response bias also applies to objects that we regularly encounter in everyday life. In conclusion, our results show that symmetry does not enhance mnemonic processes but instead induces a response bias leading individuals to judge such stimuli as having been seen.

Resume La severite de la sarcoidose est tres variable. La corticotherapie en est le traitement de reference, permettant une remission de la maladie avec un effet, cependant, purement suspensif. D’autres medicaments peuvent aussi etre efficaces : les immunosuppresseurs, les antipaludeens de synthese et l’infliximab. La moitie des patients guerissent sans traitement. Chez les autres, un traitement est necessaire d’emblee ou secondairement devant une progression de la maladie. Le traitement est administre au minimum 12 mois. Les indications d’un traitement systemique sont les atteintes ophtalmologiques graves, neurologiques, cardiaques, renales, laryngees et pulmonaires invalidantes ou evolutives, le lupus pernio et l’hypercalcemie severe. Le traitement comporte initialement de la prednisone ou de la prednisolone a 0,5 ou 1 mg/kg par jour pendant six a 12 semaines pour obtenir une reponse complete, avec decroissance secondaire par plateaux de six a 12 semaines. Apres l’arret du traitement, une surveillance de 36 mois est necessaire avant d’affirmer le diagnostic de guerison. Le methotrexate a faible dose hebdomadaire et l’azathioprine sont les immunosuppresseurs les plus utilises. Ils sont indiques en cas d’echec ou de contre-indication aux corticoides ou de corticodependance a une dose d’au moins 10 mg/j de prednisone au long cours. Le cyclophosphamide est propose en cas d’atteinte neurologique ou cardiaque severe non controlee par les traitements classiques. Les antipaludeens de synthese sont utilises dans les atteintes cutanees multifocales ou a visee d’epargne cortisonique. L’infliximab est propose en cas d’atteinte severe (sauf cardiaque) refractaire aux corticoides et aux immunosuppresseurs. Quant aux corticoides locaux, ils sont indiques en cas d’atteinte cutanee limitee, d’uveite anterieure, ou de toux. Des traitements symptomatiques peuvent etre necessaires. Le traitement de la sarcoidose pourrait etre a l’avenir individualise a partir d’une approche pharmacogenetique. La cle demeure l’acces a des traitements etiologiques et a de nouvelles molecules innovantes pour les formes resistantes aux traitements actuels.

Diffuse interstitial lung diseases (DILD) cover more than 200 conditions classified in 4 groups: secondary DILD; sarcoidosis; particular DILDs and idiopathic interstitial pneumonias. Overall, these diseases have a prevalence of 60-80/100,000 and an incidence around 30/100,000. Sarcoidosis and idiopathic pulmonary fibrosis are the 2 most frequent diseases, accounting together for more than 50% of all cases, followed by DILD related to connective tissue disease and to immunologic lung diseases. The incidence of different causes of DILD depends on epidemiologic factors: age, sex, race, smoking habits, and history. Mortality is very elevated in idiopathic pulmonary fibrosis, linked in more than 80% of cases to the fibrosis itself, with a median survival between 24 and 36 months. The epidemiologic studies on DILD have substantial limitations, and new studies must be conducted, in particular in France where we lack epidemiologic data beyond one old study of sarcoidosis and one more recent study focused on farmer's lung in the Doubs.

Subacute and chronic diffuse interstitial lung diseases Computed tomography (CT) plays an important role in all stages of management: positive diagnosis, etiological diagnosis, evaluation of lesions, ongoing monitoring, screening for complications, and prognosis. The etiological diagnosis is based on the imaging and analysis of patterns or groups of basic lesions often characteristics of a disease. Assessment of the images, the patient history, and the epidemiologic, clinical, laboratory, functional and cytologic data generally make it possible to reach a diagnosis. A pulmonary biopsy is rarely necessary. Acute diffuse interstitial lung diseases In the absence of an obvious clinical direction, CT, electrocardiography, and echocardiography are the first-line examinations to identify or rule out cardiogenic edema. CT can be used to guide bronchoalveolar lavage (BAL), widely used when the patient's respiratory condition permits. BAL can provide a diagnosis of diverse infections or help determine the cytologic type of alveolitis. CT also makes it possible to evaluate the lesions and plays a role in assessing severity. It makes it possible to choose the best sampling method and in principle directs sampling towards the most useful areas. It allows monitoring of disease course, screening of some complications, and precise localizing of tubes, drains, and catheters. Finally, it is used to assess the sequelae.

Throughout the last decades, numerous picture data sets have been developed, such as the Snodgrass and Vanderwart (1980) set, and have been normalized for variables such as name and familiarity; however, due to cultural and linguistic differences, norms can vary from one country to another. The effect due specifically to culture has already been demonstrated by comparing samples from different countries where the same language is spoken. On the other hand, it is still not clear how differences between languages may affect norms. The present study explores this issue by collecting and comparing norms on names and many other features from French Canadian speakers and English Canadian speakers living in Montreal, who thus live in similar cultural environments. Norms were collected for the photos of objects from the Bank of Standardized Stimuli (BOSS) by asking participants to name the objects, to categorize them, and to rate their familiarity, visual complexity, object agreement, viewpoint agreement, and manipulability. Names and ratings from the French speakers are available in Appendix A, available in the supplemental materials. The results show that most of the norms are comparable across linguistic groups and also that the ratings given are correlated across linguistic groups. The only significant group differences were found in viewpoint agreement and visual complexity. Overall, there was good concordance between the norms collected from French and English native speakers living in the same cultural setting.

The association of sarcoidosis and immune thrombocytopenia (ITP) has rarely been investigated. The aim of the current retrospective study was to investigate the clinical and biological phenotypes and outcome of this association in a large series of recent patients. Twenty patients (50% men) were included. Median age at sarcoidosis and ITP diagnosis was 36 (range, 10-83 yr) and 38 (range, 21-83 yr) years, respectively. In 11 of 20 (55%) patients, sarcoidosis onset preceded ITP (median interval, 48 mo; range, 6-216 mo). In 5 of 20 (25%) patients, the 2 conditions occurred concomitantly. In 4 of 20 (20%) patients, ITP onset preceded sarcoidosis (median interval, 68 mo; range, 15-153 mo). In 4 cases, sarcoidosis and ITP were not concomitant, since 1 condition was cured before the other was declared. In 12 of 20 (60%) patients there was a simultaneous onset or relapse of both ITP and sarcoidosis. Sarcoidosis phenotype was characterized by an acute onset in 40% of patients. The visceral involvement included thoracic sites in 19 of 20 (95%) patients and extrathoracic sites in 16 of 20 (80%) patients. At ITP onset, median platelet count was 11 × 10/L (range, 3-90); 17 (85%) patients had a platelet count30 × 10/L. Seven (35%) patients had a bleeding score8 without visceral bleeding.Nineteen of the 20 (95%) patients were treated specifically for ITP. After the first-line therapy (prednisone at 1 mg/kg per day for at least 3 consecutive weeks in all patients; with IVIg in addition for 10 patients with severe bleeding score), 12 of 19 (63%) patients achieved a complete response, 6 (31.5%) had a partial response, and only 1 patient failed to respond. At the end of ITP follow-up (median, 70 mo; range, 12-142 mo), 18 (90%) patients achieved a complete response, 1 achieved a partial response, and 1 had no response. After a median follow-up of 105 months, 13 of 20 (65%) patients had persistent sarcoidosis requiring prolonged therapy, and thus sarcoidosis represented the main long-term concern. Main conclusions were 1) ITP presentation was usually severe, but response to treatment was favorable in almost all cases, with no death and no severe bleeding, in contrast with older reports, 2) sarcoidosis was remarkable for the high proportion of cases with an acute onset, a chronic course, and the need for prolonged prednisone therapy, 3) sarcoidosis and ITP onset and evolution were not always synchronous.

International audience; Diffuse interstitial lung diseases (DILD) cover more than 200 conditions classified in 4 groups: secondary DILD; sarcoidosis; particular DILDs and idiopathic interstitial pneumonias. Overall, these diseases have a prevalence of 60-80/100,000 and an incidence around 30/100,000. Sarcoidosis and idiopathic pulmonary fibrosis are the 2 most frequent diseases, accounting together for more than 50% of all cases, followed by DILD related to connective tissue disease and to immunologic lung diseases. The incidence of different causes of DILD depends on epidemiologic factors: age, sex, race, smoking habits, and history. Mortality is very elevated in idiopathic pulmonary fibrosis, linked in more than 80% of cases to the fibrosis itself, with a median survival between 24 and 36 months. The epidemiologic studies on DILD have substantial limitations, and new studies must be conducted, in particular in France where we lack epidemiologic data beyond one old study of sarcoidosis and one more recent study focused on farmer's lung in the Doubs.

Subacute and chronic diffuse interstitial lung diseases Computed tomography (CT) plays an important role in all stages of management: positive diagnosis, etiological diagnosis, evaluation of lesions, ongoing monitoring, screening for complications, and prognosis. The etiological diagnosis is based on the imaging and analysis of patterns or groups of basic lesions often characteristics of a disease. Assessment of the images, the patient history, and the epidemiologic, clinical, laboratory, functional and cytologic data generally make it possible to reach a diagnosis. A pulmonary biopsy is rarely necessary. Acute diffuse interstitial lung diseases In the absence of an obvious clinical direction, CT, electrocardiography, and echocardiography are the first-line examinations to identify or rule out cardiogenic edema. CT can be used to guide bronchoalveolar lavage (BAL), widely used when the patient's respiratory condition permits. BAL can provide a diagnosis of diverse infections or help determine the cytologic type of alveolitis. CT also makes it possible to evaluate the lesions and plays a role in assessing severity. It makes it possible to choose the best sampling method and in principle directs sampling towards the most useful areas. It allows monitoring of disease course, screening of some complications, and precise localizing of tubes, drains, and catheters. Finally, it is used to assess the sequelae.