[Lung imaging in severe asthma]. / Imagerie de l'asthme sévère.

INTRODUCTION: Asthma is a common disease whose diagnosis does not typically rely on the results of imaging. However, chest CT has gained a key place over the last decade to support the management of patients with difficult to treat and severe asthma. STATE OF THE ART: Bronchial wall thickening and mild dilatation or narrowing of bronchial lumen are frequently observed on chest CT in people with asthma. Bronchial wall thickening is correlated to the degree of obstruction and to bronchial wall remodeling and inflammation. Diverse conditions which can mimic asthma should be recognized on CT, including endobronchial tumours, interstitial pneumonias, bronchiectasis and bronchiolitis. Ground-glass opacities and consolidation may be related to transient eosinophilic infiltrates, infection or an associated disease (vasculitis, chronic eosinophilic pneumonia). Hyperdense mucous plugging is highly specific for allergic bronchopulmonary aspergillosis. PERSPECTIVES: Airway morphometry, air trapping and quantitative analysis of ventilatory defects, with CT or MRI, can help to identify different morphological subgroups of patients with different functional or inflammatory characteristics. These imaging tools could emerge as new biomarkers for the evaluation of treatment response. CONCLUSION: Chest CT is indicated in people with severe asthma to search for additional or alternative diagnoses. Quantitative imaging may contribute to phenotyping this patient group.

[Endoscopic lung volume reduction for emphysema]. / Réduction de volume pulmonaire endoscopique dans l'emphysème.

Lung hyperinflation which is a hallmark of advanced emphysema plays a major role in the exertional dyspnoea experienced by patients. This has led to the development of surgical lung volume reduction which, though effective, is also associated with significant morbidity and mortality. The goal of endoscopic lung volume reduction which has developed over several years is to decrease hyperinflation without exposing patients to the risks of surgery. Several endoscopic techniques have been assessed by high quality controlled studies: airway by-pass, instillation of glue, insertion of coils or unidirectional valves, vapour ablation. The aim of this review is to present the results of these studies in terms of functional benefit and side effects. Based on these studies, an algorithm for the endoscopic management of advanced forms of emphysema is proposed.

[Idiopathic interstitial pneumonias in 2016]. / Actualités dans les pneumopathies interstitielles idiopathiques en 2016.

Idiopathic interstitial pneumonias comprise 8 clinicopathological entities, most of them with a chronic course and various prognosis. Idiopathic pulmonary fibrosis is the most frequent and most severe of these. Computed tomography has an important role for its diagnosis. It can identify the corresponding pathological pattern of usual interstitial pneumonia in about 50 percent of cases. It can suggest differential diagnosis in other cases, most frequently fibrosing nonspecific interstitial pneumonia and chronic hypersensitivity pneumonitis. Imaging features should be integrated to clinical and available pathologic data during multidisciplinary team meetings involving physicians with a good knowledge of interstitial diseases. Some cases may be unclassifiable, but these could later be reclassified as new data may occur or imaging features may change. Surgical lung biopsy is being less frequently performed and an emerging less invasive technique, lung cryobiopsy, is under evaluation. Pleuroparenchymal fibroelastosis is a distinct entity only recently described, with uncertain prevalence and prognosis that seems being quite often associated to another pattern of interstitial pneumonia.

Contribution of magnetic resonance imaging in lung cancer imaging.

Lung cancer is the leading cause of cancer death worldwide. Prognosis and treatment outcomes are known to be related to the disease stage at the time of diagnosis. Therefore, an accurate assessment of the extent of disease is critical to determine the most appropriate therapy. Currently available imaging modalities for diagnosis and follow-up consist of morphological and functional imaging. Morphological investigations are mainly performed with CT-scan and in some cases with MRI. In this review, we describe the contribution of MRI in lung cancer staging focusing on solid pulmonary nodule characterization and TNM staging assessment using chest and whole-body MRI examinations, detailing in each chapter current recommendations and future developments.

[Mediastinal fat necrosis]. / Nécrose de la graisse médiastinale.

Epicardial fat necrosis is a rare cause of benign chest pain. Its physiopathological mechanism is unknown. Diagnosis is easily performed through radiological investigations that show a round opacity of fat density limited by a dense pseudo-capsule in the anterior mediastinum, close to the heart.

Pulmonary alveolar proteinosis.

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic "crazy paving" pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy) or anti-GM-CSF antibodies (rituximab and plasmapheresis) are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.

[Pneumocystis pneumonia in a patient treated with pemetrexed for non small cell lung cancer]. / Pneumocystose chez un patient traité par pemetrexed pour adénocarcinome pulmonaire.

INTRODUCTION: Pneumocystis pneumonia is a life-threatening infection in patients undergoing chemotherapy for solid malignancies. CASE REPORT: A 49-year-old man developed gradually increasing dyspnoea while receiving pemetrexed as a third line treatment for an adenocarcinoma of the lung. The diagnosis of pneumocystis pneumonia was based on ground-glass opacities on the thoracic CT scan and alveolar lavage revealing occasional cysts of Pneumocystis jiroveci in the context of recent lymphopenia developing during chemotherapy. Treatment with cotrimoxazole for three weeks was only partially successful due to progression of the tumour. CONCLUSIONS: Pneumocystis pneumonia should be considered in cancer patients receiving antifolate drugs and presenting with increasing dyspnoea. It is important to identify a high-risk population among patients undergoing chemotherapy because of the significant morbidity and mortality and in order to administer effective prophylactic agents.

Retrosternal and pleural "bridging" fibrosis mimicking malignancy in a patient exposed to asbestos.

Mediastinal fibrosis is a rare, benign disorder characterised by proliferation of fibrous tissue within the mediastinum. It can present as diffuse mediastinal infiltration or, more frequently, as a localised soft tissue mass, usually in the right paratracheal or subcarinal areas. 18-fluorodeoxyglucose (18-FDG) positron emission tomography (PET) imaging has only been reported in a few case reports with variable aspects. Herein, we present an unusual pattern of mediastinal fibrosis, presenting as a thick retrosternal mass contiguous with pleural thickening. 18-FDG PET showed intensely increased uptake in the mass. Both the location of fibrosis and PET imaging were confusing with malignancy. Histopathological proof of benign fibrosis was obtained by surgery. The patient had been exposed to asbestos and the possible relationship of this particular pattern of fibrosis with asbestos exposure will be discussed.

Diffuse interstitial pneumonia and pulmonary hypertension: a novel manifestation of chronic granulomatous disease.

The present authors report the case of an adult with chronic granulomatous disease who developed an unusual lung fibrosis associated with severe pulmonary hypertension. Histological analysis of a lung biopsy showed a diffuse infiltration with pigmented macrophages without granulomas, which particularly involved the pulmonary arterial and venular walls. Clinical and histological findings were suggestive of pulmonary veno-occlusive disease. Such a clinical association has not been previously described in the literature and might be due to the persistent expression of gp91phox at a very low level. In conclusion, the present case report illustrates a novel manifestation of chronic granulomatous disease.

Rituximab therapy in autoimmune pulmonary alveolar proteinosis.

Idiopathic pulmonary alveolar proteinosis is presumed to be an autoimmune disorder that may lead to pulmonary insufficiency. However, steroids do not appear to be effective and the standard of therapy is whole-lung lavage. We report the first case of successful therapy with rituximab, which addresses the pathogenic mechanism of pulmonary alveolar proteinosis.

[Niemann-Pick disease type B identified following an episode of bronchopneumonia]. / Maladie de Niemann-Pick de type B révélée par une atteinte bronchopulmonaire.

Niemann Pick disease type B (NPD type B) is a rare autosomal recessive lipid storage disorder, characterized by a partial deficiency of sphingomyelinase. We report the case of an adult male patient affected by NPD type B and diagnosed at 39-years-of age. Pulmonary CT scan revealed a cranio-caudal gradient with nodular centrilobular ground glass opacities and thickening of the interlobular septa. Pathological examination of the bronchoalveolar lavage showed foamy alveolar macrophages and vacuolated bronchial epithelial cells on bronchial biopsy. Diagnostic confirmation was achieved by a decrease in cell lysosomal enzyme activity and by the presence of the homozygous DeltaR608 mutation in the acid sphingomyelinase gene (SMPD1).

[Respiratory manifestations during the course of Sjogren's syndrome]. / Manifestations respiratoires au cours du syndrome de Gougerot-Sjögren.

INTRODUCTION: Sjogren's syndrome is a common auto-immune disease. BACKGROUND: Clinically significant pulmonary involvement affects approximately 10% of patients and may be the first manifestation of the disease, putting the respiratory physician in a position to suspect and confirm the diagnosis. Besides interstitial lung disease and bronchial disorders, cough is a common symptom of the disease and particularly difficult to treat. Lung cysts and amyloid deposits, sometimes associated with lymphoma, have recently been described. The development of a primary pulmonary lymphoma, usually from MALT, is a major complication of the disease. VIEWPOINT: Characterisation of the pathophysiology of pulmonary involvement in Sjogren's syndrome and the institution of specific treatment merits the interest of the respiratory physician. CONCLUSION: The respiratory physician should consider the diagnosis of Sjogren's syndrome in many different clinico-pathological situations.

[Haemolytic uremic syndrome and acute mesenteric ischemia caused by interferon-alpha-2b in the treatment of Kaposi's sarcoma in an AIDS patient]. / Syndrome hémolytique et urémique et ischémie mésentérique aiguë lors d'un traitement par interféron alpha-2b pour une maladie de Kaposi associée au VIH.

BACKGROUND: Interferon alpha is approved for the treatment of Kaposi's sarcoma in HIV infected patients. Hemolytic and uremic syndrome (HUS) is a rare side-effect of interferon alpha and has been reported primarily in chronic myelogenous leukemia. CASE REPORT: A 44-year-old HIV-infected woman from Cameroon was admitted for treatment of cutaneous Kaposi's sarcoma. Two days later, she presented severe HUS. Abdominal pains subsequently revealed non-occlusive mesenteric ischemia. The patient rapidly improved after interferon withdrawal. DISCUSSION: To our knowledge this is the first case of HUS induced by interferon alpha given for Kaposi's sarcoma. Further, no cases of acute mesenteric ischemia have been reported with interferon alpha. It is possible that the condition may have been induced or aggravated by HUS or a low infusion rate. Interferon can exert vascular toxicity on both the mesenteric vessels and the renal vessels in a setting of microangiopathy.

Imaging appearances of metastases from neuroendocrine tumours of the pancreas.

Endocrine tumours of the pancreas (ETPs) are rare neoplasms that are frequently malignant. Despite their usual slow growth, metastases do occur and have a major impact on prognosis. Metastases may be the first manifestation of disease, and recognition of particular radiological features of these hypervascular metastases should suggest their possible neuroendocrine origin. Although somatostatin receptor scintigraphy has changed the imaging strategy for these tumours and has become their principal imaging modality, radiological techniques are still required for precise localization of scintigraphic hot spots and monitoring of response to therapy. This pictorial review shows the typical radiological features of ETP metastases and emphasizes the role of different imaging modalities.