Gene/Protein Disease Symptom Drug Enzyme Compound
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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 relationship between cigarette smoke and interstitial lung diseases (ILDs) is not clear. Respiratory bronchiolitis (RB), usually found as an incidental histologic abnormality in otherwise asymptomatic smokers, is characterized by the accumulation of cytoplasmic golden-brown-pigmented macrophages within respiratory bronchioles. A small proportion of smokers have a more exaggerated response that, in addition to the bronchiole-centered lesions, provokes interstitial and air space inflammation and fibrosis extending to the nearby alveoli. This set of histologic changes is called RB-ILD, and results in clinical symptoms. Desquamative interstitial pneumonia (DIP) is characterized by panlobular involvement, diffuse mild-to-moderate interstitial fibrosis, and massive alveolar filling with macrophages. It is well known that the histopathologic patterns of RB-ILD and DIP may overlap, and that the key features for differentiating these disorders are the distribution and the extent of the lesions: bronchiolocentric in RB-ILD and diffuse in DIP. It has been proposed that RB, RB-ILD, and DIP may be different components of the same histopathologic disease spectrum, representing various degrees of severity of the same process caused by chronic smoking, although this is still controversial. Pulmonary Langerhans' cell histiocytosis is also strongly related to cigarette smoking and is characterized by the proliferation of specific histiocytes, known as Langerhans' cells, and their infiltration of organ systems. Although RB, RB-ILD, DIP, and Pulmonary Langerhans' cell histiocytosis are considered as discrete entities of smokers, it is not infrequent to find a mixture of pathologic features rendering the histopathologic diagnosis difficult.
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PMID:Smoking-related interstitial pneumonias and pulmonary Langerhans cell histiocytosis. 1673 93

Cigarette smoking is a recognized causative agent or precipitant of specific diffuse lung diseases characterized by bronchiolar and interstitial lung inflammation. Respiratory bronchiolitis-associated interstitial lung disease and pulmonary Langerhans cell histiocytosis are now considered smoking-induced diffuse lung diseases. Desquamative interstitial pneumonia is also recognized as a smoking-induced interstitial pneumonia in most cases. These disorders affect relatively young adult smokers and may be progressive. Although distinguishable by histopathological and radiographic features, significant overlap occurs in many cases with chest radiography and lung histology showing overlapping features of smoking-related bronchiolar and interstitial lung injury. Cigarette smoking is also recognized as an important precipitant of many acute eosinophilic pneumonia cases. Smokers are at higher risk of developing fibrotic interstitial lung diseases such as idiopathic pulmonary fibrosis and rheumatoid arthritis-associated interstitial lung disease. Certain smokers also develop combined emphysema and lung fibrosis. The avoidance of primary and second-hand cigarette smoke is a critical component of management for patients afflicted with these smoking-induced diffuse lung diseases. The role of corticosteroids and other immunosuppressive treatments in the management of smoking-related interstitial lung diseases remains poorly defined and should be reserved for individuals with progressive disease despite smoking cessation. Understanding mechanisms by which tobacco induces diffuse lung pathology is critical in the pursuit of novel therapeutic approaches for these diseases.
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PMID:Diffuse lung diseases in cigarette smokers. 2300 6

Desquamative interstitial pneumonia (DIP) is characterised by the accumulation of numerous pigmented macrophages within most of the distal airspace of the lung and, sometimes, the presence of giant cells. Diagnosis of DIP is not easy and requires surgical lung biopsy. DIP is usually associated with tobacco smoke. However, the association between smoking and DIP is less robust than that with respiratory bronchiolitis with interstitial lung disease or pulmonary Langerhans' cell histiocytosis; approximately 10-42% of patients with DIP are nonsmokers. DIP can also occur in patients following exposure to certain inhaled toxins (occupational exposure) and drugs, and may occur in the context of certain viral illnesses and autoimmune diseases. In the context of DIP, occupational exposure should be systematically investigated.
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PMID:Desquamative interstitial pneumonia: an analytic review with an emphasis on aetiology. 2372 65