title: A prospective clinical multicentre study on adult pulmonary Langerhans' cell histiocytosis.
abstract: BACKGROUND
  To date the clinical picture of pulmonary Langerhans' cell histiocytosis has been described only in retrospective reports. For a better understanding, the German Scientific Study Group on the Treatment of Lung Disease (WATL) conducted an open, prospective, clinical observation study.
  
  
  METHODS
  During the period between 1994 and 2002 77 patients (40 men and 37 women) were recruited. The median observation period was 38.2 (3.2-86.7) months.
  
  
  RESULTS
  At the initial examination 50 patients were active smokers, 26 ex-smokers and 1 had never smoked. 36% of the patients showed reduced vital capacity, 28% signs of airways obstruction. On chest radiography, 74% of the patients who stopped smoking (24/50) showed regression, while 13% remained unchanged and 13% revealed progression. In the group that continued to smoke (25/50) chest radiography showed regression in 58% of cases, no change in 25% and progression in 17%. The difference was not significant, which was also true for lung function values. 3 patients died within the observation period.
  
  
  CONCLUSIONS
  The data underline the key role of smoking as the sole known risk factor. A significant effect of smoking cessation on the course could not be confirmed. The overall prognosis was good in this series as compared to previous reports.
authors: Lungenklinik Heckeshorn, HELIOS Klinikum Emil von Behring, Berlin, Germany. Schoenfeld.Berlin@T-online.de
authors: N
authors: N
authors: Schönfeld
authors: K
authors: K
authors: Dirks
authors: U
authors: U
authors: Costabel
authors: R
authors: R
authors: Loddenkemper
mesh_terms: D000328:Adult; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use*; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000188:drug therapy; D006801:Humans; D008168:Lung / Q000000981:diagnostic imaging / Q000473:pathology*; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012720:Severity of Illness Index; D013997:Time Factors; D014057:Tomography, X-Ray Computed
chemical_list: D005938:Glucocorticoids