Pulmonary histiocytosis X: evaluation with high-resolution CT.

Abstract

Eighteen patients with pulmonary lesions of histiocytosis X were studied with high-resolution computed tomography (CT). Thin-walled cysts were found in all but one patient. The other abnormalities included nodules (n = 14), cavitated nodules (n = 3), thick-walled cysts (n = 7), reticulation (n = 4), ground-glass opacities (n = 4), and irregular interfaces (n = 4). The lesions were most often diffuse (n = 16), with a topographic predominance in the upper or middle lung zones in nine patients. Comparison of CT scans and chest radiographs shows that small and large cysts and micronodules are better detected with CT. Comparison of abnormalities found in patients in the early and late stages of the disease, as well as the evolution observed in one case, suggests that CT patterns progress from nodules to cavitated nodules and thick-walled cysts to cysts to confluent cysts.

Pulmonary Lymphangioleiomyomatosis; Unusual Radiological Manifestation of Multiple Large Nodules.

“…And rarely a few small nodular opacities can be seen (7). Nodules in LCHconsist of several types of inflammatory cells including Langerhans' cells, and the majority of them measure 1 to 5 mmin diameter (5). It is suggested that the presence of these parencymal nodules is helpful to distinguish LCHfrom PLAM (6,8).…”

“…Pulmonary Langerhans cell histiocytosis (LCH) is also a rare lung disease characterized by lung infiltrates composed of Langerhans' histiocytes (3), and the radiographic appearance of LCHconsists of cystic lesions and multiple nodular opacities ranging on average from 1 to 5 mm,the largest being 10 mmin diameter (4,5). Both PLAMand LCHhave similar pulmonary cysts, which are defined as air-containing lesions surrounded by thin walls that are not shared, but in general parenchymal large nodules, when present, are helpful in distinguishing LCHfrom PLAM (6).…”

Pulmonary Histiocytosis X

“…4 High-resolution CT of the chest is superior to plain films in depicting the nodules and cysts. 5 Nodules are predominant in the early stages of the disease, and cysts-particularly in the costophrenic angles-are a later development. Pneumothorax is reported in 25% of patients.…”

Pulmonary Langerhans'-Cell Histiocytosis

“…Frequently involves lower lobes [18] Sparing of bases and costo-phrenic angles is typical [19][20][21] Biological character Clonality Invariably reported [22][23][24] Suspect reactive rather than clonal [25] …”

“…Several descriptive studies illustrate the utility of HRCT for delineating the nodules and cysts that often have a characteristic distribution in the upper and mid lung fields (Figure 3) [19,20,76].…”