[Own experience in diagnosis and therapy in pulmonary Langerhans cell granulomatosis]

We have assessed 12 patients (2 females, 10 males) aged between 19 and 53 years (mean 38.3 +/- 10.3) diagnosed with LCG during 14-year period (1985-1998). All patients were smokers. Follow-up was from 6 to 132 months (mean 47.5 +/- 44.4). LCG diagnosis was confirmed by histology in 10 cases (9 lung, and 1 bone biopsy). In 2 patients the diagnosis was made on clinical grounds, including characteristic appearance on HRCT scans. Mean FVC was 78.9 +/- 15.9% of predicted, DLCO 64.1 +/- 22% of predicted. In 8 patients (67%) FVC or DLCO were below 80% of predicted. In 2 patients with histologically proven diagnosis, HRCT was not characteristic for LCG. The treatment was introduced in 8 patients (67%). Only 3 out of 8 patients initially treated with steroids responded to this treatment. In the rest treated patients, therapy was changed to cytotoxic agents. 3 patients died (1 after 2 years, and 2 after 11 years) 2 due to LCG and 1 due to pneumonia.

HRCT appearance is not always characteristic in patients with histological diagnosis of LCG. Pulmonary involvement in LCG is frequently connected with lung function derangements. Response to steroids is poor, and switching to cytotoxic agents is often necessary.