Clinical Features of Pulmonary Langerhans Cell Histiocytosis in Korea

Abstract

Methods: The patients with PLCH confirmed by biopsy at any body site were included and the patients should have lung lesions present. A questionnaire that had items on the symptoms, lung function tests, the roentgenographic findings and the treatment was collected retrospectively at a Korean ILD Research Meeting. Results: A total of 56 cases were collected. The number of males and females was 48 and 8, respectively, and their median age was 43 years (range: 18∼67 years). The patients were current or ex-smokers in 79% of the cases. The most frequent symptom was coughing (39%), followed in decreasing order by dyspnea (38%), sputum (20%) and chest pain (20%). Pneumothorax was observed in 16 (29%) patients. Lung function tests showed a normal, restrictive, mixed or obstructive pattern in 26 (61%), 7 (16%), 7 (16%) and 3 patients (7%), respectively. Nodular-cystic lesion was most frequently observed in 59% of the patients on HRCT. The lung lesions were located in the middle and upper lobes in almost the cases. The median follow-up period was 90 months (range: 1 ∼180 months) and only two patients died during this period. Conclusion: This study provides a national survey of the patients with PLCH during a long follow-up period.