Serial computed tomography and lung function testing in pulmonary Langerhans’ cell histiocytosis

Cigarette smoking has been implicated in the development of some uncommon respiratory interstitial diseases. Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung diseases are characterized by a diffuse alveolar and peribronchiolar filling with macrophages, respectively. Pulmonary Langerhans' cell histiocytosis is a rare interstitial lung disorder characterized by the proliferation of Langerhans' cell forming interstitial infiltrates and nodules that could progress to cavitary nodules. The treatment of these disorders involves smoking cessation and sometimes the use of steroids. High-resolution computed tomography is essential for the characterization of these smoking-related interstitial lung diseases, but frequently it is necessary to create a workgroup composed by pulmonologists, pathologists, and radiologists to diagnosis and treat patients affected with these pathologies.

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Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis with different changes of pulmonary function tests

Regional blood circulation and microcirculation ◽

10.24884/1682-6655-2016-15-3-30-35 ◽

2016 ◽

Vol 15 (3) ◽

pp. 30-35 ◽

Cited By ~ 1

Author(s):

V. P. Zolotnitskaya ◽

V. I. Amosov ◽

D. V. Dsadsua

Keyword(s):

Computed Tomography ◽

Respiratory Failure ◽

Langerhans Cell Histiocytosis ◽

Pulmonary Function Tests ◽

Langerhans Cell ◽

Histiocytosis X ◽

Emission Computed Tomography ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Stage Of Development ◽

Pulmonary Microcirculation

Introduction and purpose. Disturbances of pulmonary microcirculation in patients with pulmonary Langerhans' cell histiocytosis and various types of respiratory failure are characterized by different scintigraphic patterns. Aim: to identify features of the circulatory disturbances in the lungs of patients with histiocytosis X, and various forms of bronchial conductivity changes. Material and methods: analysis of changes of microcirculation in the lungs in 54 patients with histiocytosis X, with different types of respiratory insufficiency, assessed by means of single photon emission computed tomography, perfusion scintigraphy and multislice computed tomography. Results and conclusions: in patients with histiocytosis x with obstructive type of respiratory failure, microcirculatory changes were detected at a very early stage of development of the disease. In the terminal stage of the disease, a typical scintigraphic pattern «candle flame» was observed. In patients with combined restrictive and obstructive changes (mixed type), no differences from changes of blood flow in obstructive variant were observed. In the restrictive type of respiratory failure, disorders of microcirculation do not have any typical scintigraphic pattern. The presence of characteristic nodular lesions and increasing fibrotic changes of all structural units were observed in the parenchyma of the lung.

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Pulmonary Langerhans’ cell histiocytosis

Oxford Textbook of Medicine ◽

10.1093/med/9780199204854.003.181405 ◽

2010 ◽

pp. 3446-3447

Author(s):

S.J. Bourke ◽

D.J. Hendrick

Keyword(s):

Lung Function ◽

Chest Radiography ◽

Langerhans Cell Histiocytosis ◽

Progressive Disease ◽

Langerhans Cell ◽

Cytotoxic Drugs ◽

Common Complication ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Stop Smoking ◽

Systemic Symptoms

Pulmonary Langerhans’ cell histiocytosis is characterized by a reactive monoclonal proliferation of activated histiocytes in the distal bronchioles. It presents, nearly always in smokers, with cough, breathlessness, and (sometimes) systemic symptoms. Pneumothorax (sometimes bilateral) is a common complication. Chest radiography typically shows micronodules, reticulation, and small cysts, with fibrosis in advanced cases. Patients must stop smoking. About 25% of cases resolve, 50% stabilize, and 25% lose lung function. Corticosteroids and/or cytotoxic drugs are usually given for progressive disease, but the benefits are unclear....

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