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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The ground-glass pattern is a common but nonspecific finding on CT. In certain clinical circumstances, it can suggest a specific diagnosis, indicate a potentially treatable disease, and guide a clinician to an appropriate area for biopsy. A pattern of centrilobular ground-glass nodules is fairly specific for the diagnosis of hypersensitivity pneumonitis with the appropriate clinical history. The tree-in-bud pattern indicates disease affecting the small airways. The differential diagnosis is lengthy; however, the most common process leading to this CT appearance is infection. Although commonly associated with M. tuberculosis, many infectious organisms can produce this pattern. When honeycombing is seen on HRCT, a confident diagnosis of lung fibrosis can be made. The most common causes of interlobular septal thickening on HRCT are pulmonary edema, pulmonary hemorrhage, and lymphangitic spread of cancer, and smooth thickening is characteristic of all three. Diffuse lung cysts in patients who are not immunocompromised generally signify Langerhans' cell histiocytosis, lymphangioleiomyomatosis, or centrilobular emphysema. Centrilobular emphysema can be diagnosed when the centrilobular artery is seen as a small nodular opacity in the center of the cyst. Langerhans' cell histiocytosis is often associated with parenchymal nodules, helping to distinguish it from lymphangioleiomyomatosis. When a nodular pattern is seen on HRCT, the differential diagnosis is very long, but can be narrowed by noting whether the nodules are random, centrilobular, or perilymphatic in distribution. A mosaic pattern of lung attenuation can represent an infiltrative, small airway, or vascular process. The distinction can often be made by noting the size of the pulmonary vessels in the abnormal areas of lung, and whether air trapping is present on expiratory scanning. Computed tomographic signs can be useful indicators of a specific disease process. For instance, the air bronchogram sign indicates that an opacity is intrapulmonary in location, and signals the possibility of two types of neoplasm: lymphoma and bronchioloalveolar cell carcinoma. An air crescent sign indicates recovery of the immune system in an immunocompromised patient with invasive pulmonary aspergillosis. The fallen lung sign is diagnostic of a bronchial transection in the correct clinical setting. The gloved finger sign is very suggestive of allergic bronchopulmonary aspergillosis. The halo sign is highly suggestive of early angioinvasive pulmonary aspergillosis in patients with acute leukemia. When a split pleura sign is seen, the diagnosis is often empyema, although other causes of pleuritis can lead to a similar CT appearance.
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PMID:CT signs and patterns of lung disease. 1169 64

The aim of this pictorial essay is to illustrate the distribution of normal findings and various lung diseases on coronal reconstructions as compared with cross-sectional high-resolution CT images. The volumetric CT images were obtained at 120 kVp, 200 mA/rotation, pitch 6:1, and a high-spatial-frequency reconstruction algorithm. The scans were performed using 2.5-mm collimation and reconstructed at 1.25-mm intervals. Coronal reconstructions were obtained at 2.5-mm slice thickness and 2.5-mm intervals. The pictorial includes images of normal anatomy, emphysema, idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, sarcoidosis, bronchiolitis obliterans organizing pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, lymphocytic interstitial pneumonia, pulmonary edema, tuberculosis, cytomegalovirus pneumonia, bronchiectasis, and panbronchiolitis.
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PMID:Multidetector spiral high-resolution computed tomography of the lungs: distribution of findings on coronal image reconstructions. 1236 68

Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) designates interstitial lung changes in smokers, characterized histologically by bronchiolocentric accumulation of pigmented alveolar macrophages and fibrotic or cellular inflammatory changes of pulmonary interstitium. The definition is nearly identical to that of condensate pneumopathy, smoker's pneumopathy or smoker's lung, defined by accumulation of pigmented alveolar macrophages with bland alveoloseptal or peribronchial fibrosis and cellular inflammation of the bronchial wall. In addition to respiratory bronchiolitis, which is found in nearly all smokers, RB-ILD comprises a broad spectrum of varying degrees of the interstitial reaction to the exogenous injury of inhalation smoking with gradual transition to desquamative interstitial pneumonia (DIP). In most cases RB-ILD manifestations are subclinical and detected coincidentally. Radiographic features are reticulonodular and ground glass opacities of the lung. The high resolution computed tomography reveals centrilobular nodules, ground glass opacities, thickening of bronchial walls, and in some cases a reticular pattern. Mild emphysema is frequent. Lung function analysis reveals only minor restrictive or obstructive defects in most cases, often combined with hyperinflation. CO diffusing capacity is slightly to moderately impaired. Pronounced interstitial lung diseases with serious restrictive defects and arterial hypoxemia have been reported infrequently. In differential diagnosis smoking related interstitial lung diseases (DIP, Langerhans cell histiocytosis, idiopathic pulmonary fibrosis) and other interstitial lung diseases have to be excluded. In most cases diagnosis can be achieved by bronchoalveolar lavage and transbronchial lung biopsy. In cases of pronounced interstitial lung disease or assumption of an additional interstitial lung disease besides RB-ILD a thoracoscopic or open lung biopsy can be necessary. RB-ILD has a favourable prognosis. After smoking cessation lung changes are reversible. Corticosteroid therapy is not necessary. A fatal outcome of RB-ILD has not been reported. Follow-up examinations are advisable in order to preclude other interstitial lung diseases. RB-ILD seems to be more frequent than it is assumed at present. The clinical picture is masked in most cases by the concomitant smoking induced chronic bronchitis. Thus only pronounced cases with structural changes and resulting differential diagnostic problems are diagnosed.
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PMID:[Respiratory bronchiolitis-associated interstitial lung disease (RB-ILD)]. 1278 81

Cysts and cavities are commonly encountered abnormalities on chest radiography and chest computed tomography. Occasionally, the underlying nature of the lesions can be readily apparent as in bullae associated with emphysema. Other times, cystic and cavitary lung lesions can be a diagnostic challenge. In such circumstances, distinguishing cysts (wall thickness < or = 4 mm) from cavities (wall thickness > 4 mm or a surrounding infiltrate or mass) and focal or multifocal disease from diffuse involvement facilitates the diagnostic process. Other radiological characteristics, including size, inner wall contour, nature of contents, and location, when correlated with the clinical context and tempo of the disease process provide the most helpful diagnostic clues. Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes, including coccidioidomycosis, Pneumocystis carinii pneumonia, and hydatid disease. Malignant lesions including metastatic lesions may rarely present as cystic lesions. Focal or multifocal cavitary lesions include neoplasms such as bronchogenic carcinomas and lymphomas, many types of infections or abscesses, immunologic disorders such as Wegener granulomatosis and rheumatoid nodule, pulmonary infarct, septic embolism, progressive massive fibrosis with pneumoconiosis, lymphocytic interstitial pneumonia, localized bronchiectasis, and some congenital lesions. Diffuse involvement with cystic or cavitary lesions may be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, honeycomb lung associated with advanced fibrosis, diffuse bronchiectasis, and, rarely, metastatic disease. High-resolution computed tomography of the chest frequently helps define morphologic features that may serve as important clues regarding the nature of cystic and cavitary lesions in the lung.
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PMID:Cystic and cavitary lung diseases: focal and diffuse. 1293 86

A 42-yr-old woman was referred to our hospital because of multiple small nodules in a chest radiograph. She had no symptoms such as dyspnea, cough or sputum. A chest CT revealed many centrilobular small nodules and thin-walled cysts with predominance in the peripheral area of the lungs. The specimens obtained by thoracoscopic surgery showed granulomas with scattered eosinophils and numerous Langerhans' cells. The Langerhans' cells were positive for both S-100 protein and CD1a. These findings are compatible with pulmonary Langerhans' cell histiocytosis (LCH). Since the granulomas showed no fibrotic changes, the LCH may have been in its early stages. However, there were clusters of lymphocytes and macrophages around the terminal and respiratory bronchioles, and cystic lesions without cellular infiltrates, in the specimens. The former histologic findings suggested respiratory bronchiolitis causing interstitial lung disease and the latter are indistinguishable from centrilobular emphysema. Therefore, these smoking-related diseases may have been superimposed on the LCH in this patient.
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PMID:[A case of pulmonary Langerhans' cell histiocytosis]. 1453 8

A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy.
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PMID:[Computed tomography of cystic lung diseases]. 1558 57

Emphysema, idiopathic pulmonary fibrosis and cystic fibrosis are the major indications for lung transplantation. This article will present an overview of lung transplantation in the context of rare pulmonary diseases, in particular pulmonary sarcoidosis, lymphangioleiomyomatosis (LAM), and pulmonary Langerhans Cell Histiocytosis X (LCH). It will present criteria used in deciding when a patient should be referred and will discuss medical management in the context of lung transplantation.
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PMID:Lung transplantation for rare pulmonary diseases. 1645 20

Emphysema is defined as a condition of the lung characterized by abnormal, permanent enlargement of airspaces distal to the terminal bronchiole accompanied by destruction of the alveolar walls and without obvious fibrosis. It is a very common disease with high morbidity and mortality. Histopathologically, there are two types of emphysema: panlobular emphysema, typically occurring in alpha1-antitrypsin deficiency, and centrilobular emphysema, which is strongly associated with cigarette smoking. Computed tomography (CT) allows detection of emphysema with higher sensitivity than conventional chest radiography and pulmonary function tests. CT also allows quantification of emphysema and depicts associated changes and complications. The differential diagnosis of emphysema, which is characterized by the absence of clearly definable walls on CT, includes cystic lung disease, bullae, lung laceration, Langerhans cell histiocytosis, and lymphangioleiomyomatosis -which are all characterized by visible walls on CT.
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PMID:[Imaging of emphysema]. 1722 84

Cystic lung disease is a frequently encountered problem caused by a diverse group of diseases. Distinguishing true cystic lung disease from other entities, such as cavitary lung disease and emphysema, is important given the differing prognostic implications. In this paper the features of the cystic lung diseases are reviewed and contrasted with their mimics, and the clinical and radiographic features of both diffuse (pulmonary Langerhans' cell histiocytosis and lymphangioleiomyomatosis) and focal or multifocal cystic lung disease are discussed.
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PMID:Challenges in pulmonary fibrosis. 3: Cystic lung disease. 1772 70

Cigarette smoke, a toxic collection of more than 4000 chemicals generated from combustion of tobacco plant leaves, is known to cause several respiratory ailments, including chronic bronchitis, emphysema and lung cancer, and is associated with an increase in respiratory infections. In addition, cigarette smoking is considered a principal aetiological factor responsible for the development of certain diffuse interstitial and bronchiolar lung diseases, namely respiratory bronchiolitis-interstitial lung disease (RB-ILD), desquamative interstitial pneumonia (DIP) and adult pulmonary Langerhans' cell histiocytosis (PLCH). Although not exclusively seen in cigarette smokers, substantial clinical and epidemiological data support a central role for smoking as the primary causative agent of most RB-ILD, DIP and PLCH. Additional evidence in support of cigarette smoke as a primary aetiological agent in RB-ILD, DIP and PLCH is the observation that smoking cessation may lead to disease improvement, while recurrence of these disorders has been observed to occur in the transplanted lung upon re-exposure to tobacco smoke. Furthermore, histopathological changes of respiratory bronchiolitis, DIP and PLCH (with or without co-existent emphysema) may be found on lung biopsy in the same individual, implicating smoking as a common inciting agent of these diverse lesions. Recent studies also suggest a role for cigarette smoking as a potential co-factor in the development of acute eosinophilic pneumonia, usual interstitial pneumonia and rheumatoid arthritis-associated interstitial lung disease. In the current review, we propose a novel classification that takes into account the complex relationship between cigarette smoking and diffuse lung diseases. Investigation on the role of smoking as a potential causative factor or modifier of these diverse diffuse lung diseases is important, as smoking cessation utilizing state-of-the-art tobacco cessation efforts should be a central part of therapy, while pharmacotherapy with corticosteroids or other immune modifying agents should be reserved for selected patients.
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PMID:Cigarette smoking and diffuse lung disease. 1862 8


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