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BackgroundInhalation of crystalline silica (cSiO2) is associated with rheumatoid arthritis (RA). Research on cSiO2 has historically focused on professional exposures and on male workers. However, cSiO2 is ubiquitously present in the environment, substantial exposure can take place in both men and women, in other professional activities and even beyond occupational context.ObjectivesTo identify the main sources of exposure to crystalline silica in a series of RA patients not selected based on their professional activity, and to assess the association between silica exposure and disease features.MethodsThe Dust Exposure Life-Course Questionnaire (DELCQ) is a novel tool that longitudinally quantifies both occupational and non-occupational lifetime exposure to crystalline silica. The DELCQ was previously validated in a representative sample of the general French population that serves as control source for studies in specific diseases. The DELCQ was administered to 97 consecutive patients with RA, exposure scores were compared between cases and age-, sex- and smoking status-matched controls (1:4). The main sources of silica exposure were identified in cases and controls and source-specific exposure levels compared. The association between DELCQ scores and disease variables in cases was tested at uni- and multivariable analysis.ResultsIn women with RA, the main sources of crystalline silica exposure were: cleaning activities, dusty clothes laundry and talcum powder handling, with higher exposure levels from these sources vs. the general population (pConclusionCleaning activities, dusty clothes laundry and talcum handling are underestimated sources of crystalline silica exposure that are overrepresented in women with RA compared to the general population and may contribute to the pathogenesis of the disease.Table 1.Association between occupational silica exposure (by quartile) and HRCT lung abnormalitiesHRCT abnormalityOES(Quartiles)UnadjustedOR (95% CI)AdjustedOR (95% CI)Mediastinal lymphadenopathyQ1-Q3(ref)(ref)Q44.1 (1.2 to 13.9)6.3 (1.4 to 27.7)Interstitial lung diseaseQ1-Q3(ref)(ref)Q46.3 (1.5 to 26.6)6.5 (1.3 to 32.6)EmphysemaQ1-Q3(ref)(ref)Q46.4 (1.6 to 24.7)2.9 (0.6 to 13.7)Abbreviations: OES occupational exposure score. Adjusted OR: adjusted on smoking, sex, disease duration, ACPA and RF status.Disclosure of InterestsNone declared.

OBJECTIVE: Interstitial lung diseases (ILDs) are associated with poor prognosis in the intensive care unit (ICU). We aimed to assess factors associated with hospital mortality in ILD patients admitted to the ICU and to investigate long-term outcome.MATERIAL AND METHODS: This was a retrospective study in a teaching hospital specialised in ILD management. Patients with ILD who were hospitalised in the ICU between 2000 and 2014 were included. Independent predictors of hospital mortality were identified using logistic regression.RESULTS: A total of 196 ILD patients were admitted to the ICU during the study period. Overall hospital mortality was 55%. Two years after ICU admission, 70 (36%) patients were still alive. Of the 196 patients, 108 (55%) required invasive mechanical ventilation, of whom 21 (20%) were discharged alive from hospital. Acute exacerbation of ILD and multi-organ failure were highly associated with hospital mortality (OR 5.4, 95% CI 1.9–15.5 and OR 12.6, 95% CI 4.9–32.5, respectively).CONCLUSION: Hospital mortality among ILD patients hospitalised in the ICU was high, but even where invasive mechanical ventilation was required, a substantial number of patients were discharged alive from hospital. Multi-organ failure could lead to major ethical concerns.

AIM To compare combined 2-[18F]-fluoro-2-deoxy- d- glucose (FDG)-positron-emission tomography (PET) and cardiac magnetic resonance imaging (CMR) for the diagnosis and therapy monitoring of cardiac sarcoidosis (CS). MATERIALS AND METHODS Eighty patients with sarcoidosis and a suspicion of CS who underwent PET and CMR were included retrospectively. PET was undertaken after a low-carbohydrate–high-fat diet in all patients using a combined 16-section PET/computed tomography (CT) camera. PET was considered positive (PET+) in cases of focal or multifocal FDG uptake. CMR was considered positive (CMR+) in cases of subepicardial late gadolinium enhancement (LGE). A subgroup of 50 patients (50/80) was monitored during therapy and classified as responders or non-responders. RESULTS Eighty-two percent of patients with PET+ (9/11) also had CMR+ imaging, with good spatial agreement (kappa=0,79; 95% confidence interval [CI]: 0.65–0.94). Twenty-seven percent (22/80) had residual physiological FDG uptake, with a standardised uptake value (SUV) not significantly different compared to the SUV from pathological uptake (6.4 versus 6 respectively, p=0,92). The clinical response was more frequent in patients with baseline PET+ compared to baseline PET– (80% versus 45%, p=0.07). PET findings improved in all cases under treatment (7/7), whereas LGE improved in only 33% of patients (3/9). CONCLUSION Due to high risk of false-positive or undetermined findings, PET might be performed as a second-line study in cases of LGE, to assess inflammatory load. In addition, PET seems suitable to predict and assess response under therapy.

Anti-PD1 immunotherapies have become an essential treatment for bronchial cancer. According to published studies, PD1 and PD-L1 inhibitors have a better toxicity profile than chemotherapy. Nevertheless, some immune related toxicities can be potentially severe, such as induced interstitial lung disease (ILD). Currently, ILD patients are excluded from clinical trials using immunotherapy in lung cancer. IPF is the most frequent and severe form of ILD. Lung cancer represents a major complication of this disease and to date few data exist on the safety of immunotherapy in this context. We report 3 cases of IPF with lung cancer treated by nivolumab. All had a clinically mild to moderate IPF. The patients had received at least one line of chemotherapy before nivolumab and had progressive, metastatic lung cancer. Two patients experienced rapid cancer progression without immune toxicities. The third had a partial response but developed grade III immune colitis that led to discontinuation of the treatment. None developed lung toxicity or worsening of IPF on CT during follow-up, and death was always related to progression of the cancer. In our series of three patients with IPF, nivolumab was well tolerated with regard to their pulmonary condition. As inflammation and autoimmunity are probably marginal mechanisms in the pathogenesis of IPF, we do not believe that the presence of IPF should definitely disqualify potential candidates for treatment with nivolumab. Decisions should be taken, case-by-case, in selected patients without severe IPF and with no evidence of autoimmunity. In view of the epidemiology of lung cancer in IPF and the critical role of immunotherapy in the management of lung cancer, studies of prospective cohorts are urgently needed in this population.

To describe the residual broncho-pulmonary lesions and evaluate the role of CT scanning at the end of treatment of pulmonary tuberculosis.Analysis of the initial and end of treatment CT scans of 56 patients with pulmonary tuberculosis according to a reading grid including parenchymatous and airways lesions. The CT data at the end of treatment were analysed in relation to the clinical and microbiological data, and the original CT scan.Active lesions (thick walled cavities and/or centrilobular micronodules) persisted in 24 patients (43%) after a mean treatment period of 7 months. The persistence of these signs of activity was correlated with the initial presence of a cavitary syndrome (p=0.027), with predominant sub-segmentary bronchial involvement, with extensive micronodular spread (p=0.024) and with bronchiectasis (p=0.04). These residual lesions were not associated with an increased risk of relapse.The persistence of signs of activity on the CT scan at the end of treatment of tuberculosis do not necessarily correspond to an absence of cure but to a radiological delay. This imaging is nevertheless useful to make an assessment of any subsequent changes in the bronchial tree and to estimate the risk of later complications.

Bronchial thermoplasty is a recent endoscopic technique for the treatment of severe asthma. It is an innovative treatment whose clinical efficacy and safety are beginning to be better understood. Since this is a device-based treatment, the evaluation procedure of risks and benefits is different that for pharmaceutical products; safety aspects, regulatory requirements, study design and the assessment of the magnitude of effects may all be different. The mechanism of action and optimal patient selection need to be assessed further in rigorous clinical and scientific studies. This technique is in harmony with the development of personalised medicine in the 21st century. It should be developed further in response to the numerous challenges and needs not yet met in the management of severe asthma.

Introduction Le stade IV de la sarcoidose, stade de fibrose pulmonaire presente un pronostic pejoratif [1] . La decision therapeutique a ce stade est difficile a prendre. L’hypothese de notre etude etait que le TEP scanner avait une valeur pronostique en differenciant les lesions inflammatoires des lesions fibreuses. L’objectif principal etait de rechercher si la fixation du parenchyme pulmonaire au TEP scanner etait predictive de l’evolution fonctionnelle respiratoire, et d’etudier sa valeur ajoutee par rapport a la tomodensitometrie (TDM) thoracique. Patients et methodes Etude retrospective, monocentrique, incluant des patients atteints de sarcoidose de stade IV. Au bilan initial, un TDM thoracique, un TEP scanner et une exploration fonctionnelle respiratoire (EFR) etaient realises. L’activite etait evaluee pour le TDM par le score d’Oberstein [2] . L’activite au TEP scanner etait evalue par le SUVmax et l’etendue de la fixation du parenchyme pulmonaire (0 : absence de fixation, 1 : fixation Les patients recevaient ou non un renforcement therapeutique. L’EFR de suivi etait de 6 a 12 mois. L’amelioration des EFR etait caracterisee par l’augmentation de ≥ 10 % de CVF, et/ou ≥ 15 % DLCO et/ou ≥ 10 % VEMS. Compte tenu du caractere retrospectif de l’etude, le resultat du TEP scanner a pu etre utilise pour prendre une decision therapeutique. Pour se soustraire du facteur traitement, des analyses en regression ont ete effectuees. Resultats Cinquante et un patients ont ete inclus. Une analyse en regression lineaire multivariee a permis de demontrer la valeur pronostique de l’extension de la fixation (R2 = 0,32 ; p = 0,002) et du score d’activite TEP (R2 = 0,32 ; p Conclusion Dans cette etude le TEP scanner est predictif d’une amelioration des EFR independamment de la prise en charge therapeutique dans la sarcoidose de stade IV et donc de la reversibilite des lesions. Il pourrait avoir une place centrale dans l’evaluation de l’activite granulomateuse residuelle et aider le clinicien dans sa decision therapeutique en appreciant l’extension et l’intensite de la fixation pulmonaire notamment grâce au score d’activite TEP. Nos resultats doivent etre confirmes par d’autres travaux prospectifs aux plus grands effectifs.

Pneumocystis jirovecii is the only fungus of its kind to be pathogenic in humans. It is primarily responsible for pneumonia (PJP). The key to understanding immune defences has focused on T-cells, mainly because of the HIV infection epidemic. Patients presenting with PJP all have a CD4 count below 200/mm(3). The introduction of systematic primary prophylaxis and the use of new anti-retroviral drugs have significantly reduced the incidence of this disease in the HIV-infected population, mainly in developed countries. The increasingly frequent use of corticosteroids, chemotherapy, and other immunosuppressive drugs has led to an outbreak of PJP in patients not infected by HIV. These patients presenting with PJP have more rapid and severe symptoms, sometimes atypical, leading to delay the initiation of a specific anti-infective therapy, sometimes a cause of death. However, the contribution of new diagnostic tools and a better understanding of patients at risk should improve their survival.

Vascular manifestations of Cogan's syndrome are rarely reported. We report the case of a young woman followed for typical Cogan's disease. Serious vascular involvement was found only during work-up for arterial hypertension. This case highlights potentially asymptomatic nature of extensive vasculitis affecting large and medium-sized vessels in Cogan's disease. Careful screening is required to prevent life-threatening complications.

Computed tomography is important for the diagnosis and follow-up of chronic diffuse interstitial lung diseases. Image quality has improved from each generation of scanner to the next and this continues to allow a better characterization of extent of pathology, or even the nature of the pathological process (potentially reversible inflammatory lesions compared to fibrotic lesions). The diagnostic imaging approach has evolved at the same time as technological developments. We initially thought in terms of the predominant lesions (nodular, alveolar consolidation, ground-glass opacity), and then moved to reasoning based on patterns, which are a combination of several elementary lesions (typically for the diagnosis of idiopathic pulmonary fibrosis). Nowadays, studies are focused on building models characterizing a specific disease and which combine several distinct patterns (typically for ground-glass opacity analysis). CT also allows a quantification of the extent of lung disease, which is linked to the prognosis of the disease and helps to monitor its progression. This quantification is usually based on visual criteria, the principles of which are summarized here. The development of automated quantification software could in the near future, be a support for the radiologist.

Sarcoidosis is a juvenile systemic granulomatosis. Its polymorphic clinical presentation depends on its different localisations, thoracic and extrathoracic. The role of imaging is very important for all localisations; but for mediastinopulmonary involvement, which is the most frequent (90% of cases), it plays a major role in detecting the disease, diagnosing it, its prognosis, decision-making regarding treatment of it and in the monitoring of its development. Standard radiography, which sometimes detects the disease, forms the basis for its four-stage prognostic classification. CT scanning enables the study of mediastinal and hilar lymphadenopathy and the study of parenchyma, making it possible to identify micronodules of lymphatic distributions, alveolar opacities, septal lines, ground-glass hyperintensities, nodules surrounded by a ring of satellite micronodules, peribronchovascular thickening; all potentially reversible lesions. Elsewhere, it highlights irreversible fibrous lesions: hilar peripheral linear opacities; septal linear opacities; bronchial distortion, honeycomb destruction or even perihilar fibrotic masses. Less frequently we can visualise bronchiolar or cystic involvement. Benign in most cases, the sarcoidosis prognosis becomes bleaker in the event of hemoptysis, Aspergillus colonisation or before the onset of pulmonary hypertension.

Management of chronic obstructive pulmonary disease (COPD) has made considerable progress over the last 15 years, with the development of pulmonary rehabilitation, new molecules to facilitate smoking cessation, and several medical treatments. Many therapeutic needs, however, remain to be met.Several lines of research on inflammation and COPD are promising, and some will probably result in new treatments. These may target specific populations, identified by clinical phenotype or by biomarkers. The forthcoming arrival of iPDE-4s on the market illustrates how knowledge of inflammation and remodeling and of some of the underlying mechanisms finally, after many years' development, has broadened the range of treatments available to help improve patients' daily life and outcomes.The availability of such treatments, however, does not mean that knowledge of the disease in the general population and among healthcare workers can be neglected. Early detection (at a stage when treatment can already be effective) and patient education which promotes therapeutic compliance and lasting lifestyle change need to be developed further.

The objective of the present article is to review available data on possible links between phenotypes and inflammatory profiles in patients with chronic obstructive pulmonary disease (COPD).Chronic bronchitis is associated with proximal bronchial inflammation and small airway inflammation with remodeling at the site of obstruction. CT scanning enables patients to be phenotyped according to the predominantly bronchial or emphysematous nature of the morphological abnormality. Exacerbations, in a context of persistently elevated baseline inflammation, are associated with increased inflammation and a poor prognosis. Long-term studies have correlated inflammatory markers (and anti-inflammatory drug effects) with dynamic hyperinflation, possibly confirming that inflammation promotes hyperinflation. The inflammatory cell count in the pulmonary arterial walls correlates with the severity of endothelial dysfunction. The risk of developing pulmonary hypertension would seem to increase with low-grade systemic inflammation. The role of low-grade systemic inflammation in COPD co-morbidities, and in nutritional and muscular involvement in particular, remains a matter of debate. Regular physical exercise may help reduce this inflammation.In COPD, many aspects of the clinical phenotype are related to inflammation. Better knowledge of these relationships could help optimize current and future treatments.

The present study reviews the literature on inflammation and remodelling mechanisms in chronic obstructive pulmonary disease (COPD). The development of COPD is associated with chronic pulmonary inflammation. Immunity (innate or adaptive) plays a role in its onset and continuation. Airways inflammation alters bronchial structure/function relations: increased bronchial wall thickness, increased bronchial smooth muscle tone, seromucosal gland hypersecretion and loss of elastic structures. Circulating markers of pulmonary inflammation indicate its systemic dissemination. Oxidative stress plays a major role in the onset and persistence of tissue abnormalities. The determinants of extra- and intra-cellular redox control are only partially known. Susceptibility genes, antioxidant system insufficiency and reduced levels of anti-age molecules and of histone deacetylation are also involved. The molecular and cellular targets of inflammation and remodelling are numerous and complex. Currently, tools exist to limit inflammation in COPD but not to act on structural remodelling.

Connective tissue disorders correspond to a heterogeneous group of inflammatory diseases characterized by abnormal immune system activity leading to connective tissue alterations in multiple parts of the body. In adults, connective tissue disorders include rheumatoid arthritis, progressive systemic sclerosis, Sjögren syndrome, systemic lupus erythematosus, dermatomyositis and polymyositis, ankylosing spondylitis, and mixed connective tissue disease. Broncho-pulmonary involvement may be variable with involvement of all anatomical components of the lung. Involvement of other intrathoracic structures (pleura, respiratory muscles, heart, rib cage) is frequent. The most specific manifestations include interstitial lung diseases and pulmonary hypertension. During follow-up, progressive respiratory diseases may occur due to the treatment, infections, pulmonary embolism or neoplasms.

Multidetector row computed tomography (MDCT) is the imaging modality of reference for the diagnosis of bronchiectasis. MDCT may also detect a focal stenosis, a tumor or multiple morphologic abnormalities of the bronchial tree. It may orient the endoscopist towards the abnormal bronchi, and in all cases assess the extent of the bronchial lesions. The CT findings of bronchial abnormalities include anomalies of bronchial division and origin, bronchial stenosis, bronchial wall thickening, lumen dilatation, and mucoid impaction. The main CT features of bronchiectasis are increased bronchoarterial ratio, lack of bronchial tapering, and visibility of peripheral airways. Other bronchial abnormalities include excessive bronchial collapse at expiration, outpouchings and diverticula, dehiscence, fistulas, and calcifications.

To describe the high resolution CT (HRCT) imaging and functional features of the emphysema/fibrosis syndrome.A total of 61 patients were included based on HRCT. We have quantified the extent of fibrosis and emphysema lesions and a combined score was calculated. The scores were correlated to pulmonary function test parameters and specific HRCT features were described.The emphysema and fibrosis scores correlated with functional parameters of obstruction and restriction respectively. The combined score correlated with the reduction in DLCO and degree of pulmonary hypertension. Three HRCT patterns were identified: progressive transition (n=23, 38%) with diffuse emphysema (centrilobular and/or bullous) and zone of transition between bullae and honeycombing; paraseptal emphysema (n=13, 21%) with predominant subpleural bullae of enlarging size at the bases; separate processes (n=14, 23%) with independent areas of fibrosis and emphysema. Eleven patients (18%) could not be classified. The HRCT imaging features changed based on TLC (p=0.04) and FEV1/FVC (p=0.01).The emphysema/fibrosis syndrome may be associated with different patterns on HRCT corresponding to specific profiles on pulmonary function tests.

Sarcoidosis is a granulomatous disease of unknown etiology. Lung and lymphatic system are the principal localisations. Clinical presentations are various depending on involved organs. Some presentations, which are easily diagnosed, are typical and frequent. Atypical forms have unusual presentations and/or are rare. Beside, in a multisystemic sarcoidosis, the affection of only one organ can be unusual. Rigorous diagnosis procedure could avoid errors.Twenty percent of sarcoidosis have atypical presentation. However, each of them are infrequent. Atypical features are wide and can concern pulmonary or extrapulmonary manifestations, general manifestations, blood testing or pathological pattern.Describing atypical forms are necessary for their diagnosis. The diagnosis of atypical sarcoidosis is found on the knowledge of atypical forms previously described, presence of granulomas on specimen biopsy and excluding other granulomatous disease.

Malignant tumors of the pleura are most often diffuse, nethertheless they are sometimes localized. There is an overlap of the radiologic features of the benign and malignant pleural lesions. The differential diagnosis may be difficult, even on histological sample. Imaging allows the diagnosis of pleural involvement, suggests the malignity, guides percutaneous or thoracoscopic biopsies of the pleura, defines extent of the tumor and follows the course of the disease. We will describe the malignant pleural tumors: pleural metastases, pleural involvement of broncho-pulmonary cancer, of lymphoma and leukaemia. Then the rare pleural tumors will be described: malignant pleural fibroma, sarcoma, histiocytoma and hemangiopericytoma.

Introduction Les stenoses laryngo-tracheales dans les pemphigoides des muqueuses (PM) sont mal connues. Nous rapportons une serie de 11 cas de PM avec stenose laryngo-tracheale. Materiel et methodes Cette etude retrospective porte sur les patients suivis pour une PM dans notre centre du 01/01/2005 au 30/06/2012. Tous les patients ayant des symptomes ORL et/ou une atteinte OPH severe ont eu au moins une endoscopie nasolaryngee. Si une stenose laryngee/tracheale etait notee, les cas etaient inclus dans l’etude. Resultats Onze cas (3 % de l’ensemble des PM) dont 4 epidermolyses bulleuses acquises (EBA) ont ete inclus. Tous avaient une PM severe caracterisee par ≥ 3 sites atteints (n = 10) et/ou 1 fibrose conjonctivale severe (≥ stade IIIA de Foster ; n = 3) et/ou 1 atteinte œsophagienne (n = 3). Le delai moyen entre les diagnostics (dg) de PM et de stenose etait de 5,8 ans. Lors de la derniere visite avant le dg de stenose, 3 patients etaient en remission quasi complete, 1 controle, 5 non controles et 2 en rechute. Lors du dg de stenose, etaient notes des symptomes larynges (n = 5, dyspnee/dysphonie : 4/4) et/ou ORL autres (n = 9) ou aucun symptome (n = 2). Les traitements en cours etaient dapsone (n = 8), immunosuppresseurs classiques (n = 2) et rituximab (RTX) (derniere perfusion Discussion Il s’agit de la plus grande serie de stenoses tracheo-laryngees dans les PM. Celles-ci sont tres rares et surviennent tardivement au cours de PM severes, notamment dans les EBA (36,4 % de notre serie). Elles sont le plus souvent asymptomatiques (55 %) et supra-glottiques (82 %), inconstamment objectivees par le scanner (73 %) et les EFR (62 %). La majorite des cas est amelioree ou stabilisee par les traitements systemiques (72 % des cas). Conclusion Ces resultats indiquent l’importance d’un suivi au long cours des PM severes avec des examens ORL reguliers associes a un scanner et des EFR pour le depistage et la surveillance des stenoses laryngo-tracheales, meme en l’absence de signe clinique tracheo-larynge. Le traitement systemique pourrait permettre d’eviter la tracheotomie.

Pulmonary embolism (PE) is a common condition with considerable morbidity and mortality [1]. Prompt and accurate diagnosis is important because the mortality of untreated pulmonary embolism is high and serious complications can occur with its treatment, long-term anticoagulation [2]. Because there are no specific signs or symptoms of this condition, the diagnosis relies on imaging tests. In the early 1990s, the introduction of spiral computed tomography (CT) technology dramatically modified the evaluation of pulmonary arteries, enabling a direct insight into endovascular abnormalities and thus a direct depiction of endoluminal clots. Successive technological advances have reinforced its diagnostic impact, explaining why this imaging tool is now included in the noninvasive diagnostic algorithms proposed in routine clinical practice. The purpose of this article is to focus on multislice spiral CT angiography in the management of thromboembolic disease.