title: Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X.
abstract: 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).
authors: Lungenklinik Heckeshorn, Universitätsklinikum Rudolf Virchow, Berlin, BRD.
authors: N
authors: N
authors: Schönfeld
authors: W
authors: W
authors: Frank
authors: S
authors: S
authors: Wenig
authors: P
authors: P
authors: Uhrmeister
authors: E
authors: E
authors: Allica
authors: H
authors: H
authors: Preussler
authors: A
authors: A
authors: Grassot
authors: R
authors: R
authors: Loddenkemper
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis / Q000188:drug therapy; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology; D008171:Lung Diseases / Q000175:diagnosis / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use; D011859:Radiography; D011877:Radionuclide Imaging; D012129:Respiratory Function Tests
chemical_list: D011241:Prednisone