대한흉부영상의학회 Korean Society of Thoracic Radiology GuerBet

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대한흉부영상의학회 Weekly Case 검색
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Weekly Chest CasesArchive of Old Cases

Case No : 32 Date 1998-06-08

  • Courtesy of Mi-Young Kim,M.D. / Sejong General Hospital
  • Age/Sex 28 / M
  • Chief Complaintdyspnea
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4

Chest PA

Diagnosis With Brief Discussion

Diagnosis
Pulmonary Histiocytosis X
Radiologic Findings
The pathogenesis of the pulmonary lesions of histiocytosis X is poorly understood. HX exhibited cysts scattered throughout the lung parenchyma. In almost all patients, HRCT demonstrates cystic air-spaces, which are usually less than 10 mm in diameter; these cysts are characteristic of HX. On HRCT, the lung cysts have walls that range from being barely perceptible to being several millimeters in thickness. The presence of distinct walls allows diffrentiation of these cysts from areas of emphysema. Although many cysts appear round, they can also have bizarre shapes, being bilobed, clover-leaf shaped, or branching in appearance. Confluent or conjoined cysts with persisting septations are seen in more than two thirds of patients. HRCT findings closely mirror the gross pathologic appearances of this disease. The cysts were lined with fibrous tissue which interrupted focally by a diverse population of inflammatory cells and histiocytes. Interstitial cellular infiltration is associated with destruction and retraction of the lung parenchyma, alterations which appeared to result in the formation of new cystic spaces. The Langerhans' histiocyte may play a role in the production of fibrous tissue in the early formation of cyst walls. The mechanism of cystic changes is uncertain. Patterns of the nodules or cysts may represent longevity of the lesions. Early stage of the disease is characterized by the abundance of nodules and cavitated nodules, whereas cysts are few and do not tend to be conluent. Advanced disease is characterized by a small number of nodules, whereas cysts have increased in number and size and are confluent. The following sequence of abnormalities seen with CT corresponds to the evolution of the disease: nodules, cavitated nodules, thick-walled cysts, cysts, and confluent cysts, rupture or collapse of cysts with or without pneumothorax.
Brief Review
References
Keywords
Lung, Interstitial lung disease, LCH, smoking related ILD, Pulmonary Histiocytosis X

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