Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X

Respiration. 1993;60(1):38-44. doi: 10.1159/000196171.


Clinical appearance, radiologic findings, lung function and results of corticosteroid therapy were analyzed in 42 adult cases of biopsy-proven pulmonary histiocytosis X. Symptoms were present in only 64%. Using the ILO classification 1980, the evaluation of chest radiographs revealed all categories of profusion, size and shape of nodules. The characteristic 'ring figures', i.e. thin-walled cysts, in the parenchyma were detected in 78% of conventional tomographies (n = 27) and in all patients examined by CT (n = 5). In patients with early disease, lung function tests (n = 26) including body plethysmography, pulmonary diffusing capacity and ergospirometry revealed that parameters of gas exchange are most sensitive (TL,CO 84%, KCO 72%). Bronchial reactivity to carbachol was significantly higher than in controls (n = 12). Lung perfusion scintigram showed an abnormal, but uncharacteristic pattern in 81% (n = 26). During corticosteroid therapy, no progression was observed (n = 36). 85% of patients with radiographic evidence of progressive disease improved after administration of corticosteroids (n = 14).

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Female
  • Histiocytosis, Langerhans-Cell* / diagnosis
  • Histiocytosis, Langerhans-Cell* / drug therapy
  • Humans
  • Lung / diagnostic imaging
  • Lung / pathology
  • Lung Diseases* / diagnosis
  • Lung Diseases* / drug therapy
  • Male
  • Middle Aged
  • Prednisone / therapeutic use
  • Radiography
  • Radionuclide Imaging
  • Respiratory Function Tests


  • Prednisone