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.
Pulmonary Langerhans Cell Histiocytosis X
