(Eosinophilic granuloma, pulmonary granulomatosis X; Pulmonary Langerhans cell granulomatosis, histiocytosis X)

Pulmonary Langerhans cell histiocytosis (PLCH) is defined as proliferation of monoclonal Langerhans cells in the interstitium and in the airways of the lungs. The etiology is unknown, but cigarette smoking plays a primary role. The symptoms is to dyspnea, coughing, fatigue and pleuritic chest pain. Diagnosis is based on history and imaging, and sometimes on BAL and biopsy. Treatment includes smoking cessation. Corticosteroids are often given, the benefits are unclear. Lung transplantation is curative in combination with smoking cessation in general. The 5-year survival rate is 74%. Patients have an increased risk of cancer.

(See also Overview of interstitial lung disease)

Pulmonary Langerhans cell histiocytosis (PLCH) is defined as proliferation of monoclonal Langerhans cells in the interstitium and in the airways of the lungs. The etiology is unknown, but cigarette smoking plays a primary role. The symptoms is to dyspnea, coughing, fatigue and pleuritic chest pain. Diagnosis is based on history and imaging, and sometimes on BAL and biopsy. Treatment includes smoking cessation. Corticosteroids are often given, the benefits are unclear. Lung transplantation is curative in combination with smoking cessation in general. The 5-year survival rate is 74%. Patients have an increased risk of cancer. (See also Overview of interstitial lung disease) PLCH is a disease in which monoclonal CD1a + Langerhans cells (a Histiozytenart), accompanied by lymphocytes, plasma cells, neutrophils and eosinophils infiltrate the bronchioles and the alveolar interstitium. PLCH is a manifestation of Langerhans cell histiocytosis, the organs insulated can infect the same time (most commonly lung, skin, bone, pituitary and lymph nodes) or. PLCH occurs in ? 85% of cases in isolation. The etiology of PLCH is unknown, but the disease occurs almost exclusively in white smokers aged between 20 and 40 years. Men and women are equally affected. Women develop the disease later, but the age differences between the sexes at the beginning of the outbreak may represent differences in smoking behavior. The pathophysiological changes include recruitment and proliferation of Langerhans cells in response to cytokines and growth factors that are secreted by alveolar macrophages caused by cigarette smoking. Symptoms and complaints Typical symptoms and complaints from PLCH are dyspnea, nonproductive cough, fatigue, fever, weight loss and pleuritic pain. 10-25% of patients suffer a sudden, spontaneous pneumothorax. About 15% of patients are asymptomatic, and the disease is diagnosed as radiological incidental finding. Pain caused by bone cysts (18%), rash (13%) and polyuria resulting from diabetes insipidus (5%) are the most common manifestations of extrapulmonary involvement. They occur in up to 15% of patients, but rarely the main symptom of PLCH. There are few PLCH typical examination findings; usually there is a normal finding. Diagnosis High-resolution CT (HRCT) lung function tests Sometimes bronchoscopy and biopsy A PLCH is suspected based on history and chest x-ray findings and confirmed by HRCT and bronchoscopy with biopsy and BAL. The chest x-ray image typically shows a double-sided symmetrical nodular drawing in the middle and high fields of cystic changes and normal or increased lung volumes. The basal parts of the lungs are often left out. The findings may be similar to the case of chronic obstructive pulmonary disease (COPD) or at a lymphangioleiomyomatosis. The confirmation in HRCT with detection of (often oddly shaped) cysts in the middle and upper lobes and / or nodules with interstitial thickening is considered to be diagnostic of PLCH. The findings in lung function tests are normal, restrictive, obstructive or mixed, depending on at which time carried out the investigation in the disease process. Most often, the CO diffusing capacity (DLCO) is reduced and the load capacity limited. Pulmonary Langerhans cell histiocytosis Image courtesy of Harold R. Collard, M.D. var model = {thumbnailUrl: ‘/-/media/manual/professional/images/langerhans_cell_histiocytosis_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/-/media/manual/professional/images/langerhans_cell_histiocytosis_high_de.jpg?la = en & thn = 0 ‘, title:’ Pulmonary Langerhans cell histiocytosis ‘description:’ u003Ca id = “v37893186 ” class = “”anchor “” u003e u003c / a u003e u003cdiv class = “” para “” u003e u003cp u003eDie high-resolution CT scan through the upper lobe shows nodules and irregular airtight cysts. These findings are characteristic of Langerhans cell histiocytosis in a patient with cigarette smoking history of u003c / p u003e u003c / div u003e ‘credits’. Image courtesy of Harold R. Collard

Health Life Media Team

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