Pulmonary Lungerhans cell granulomatosis is a rare disease with variable clinical findings and uncertain prognosis.The aim of our study was to prospectively evaluate the clinical picture of pulmonary Langerhans cells granulomatosis in patients admitted to our Department in 2000–2008.
Starting January 2000 all patients with pulmonary Langerhans cell granulomatosis (PLCG) on the basis of symptoms, chest X-ray, HRCT criteria and BAL findings were enrolled in the study.
Since 1. 1. 2000 to 31. 12. 2008 12 patients have been enrolled, 6 males and 6 females with average age 41 ± 17 yrs, 11 smokers and one non-smokers (0.22% of all patients). The most common symptoms were exertional dyspnoe and cough. A mild obstructive ventilatory disorder (FEV1 74.1 ± 17.6 pred) with moderate reduction of TLco (53.8 ± 17.3 pred) was observed. In all cases the diagnosis of PLCG was suspected by HRCT scans and confirmed by BAL immunocytology (CD1+cells 32.9 ± 13.6%), S-100 protein positive cells in all cases. Smoking abstinence and in 7 cases prednisolone were therapeutic modalities. Unfavorable course of PLCG was observed in three cases.
PLCG is according to our experience a rare disease without male predominance which may rarely affect also non-smokers. HRCT scans are highly suggestive of diagnosis which may be confirmed by BAL immunocytology. The lung biopsy is necessary in the minority of cases.
With increased proportion of female smokers in the Czech Republic the PLCG may be observed with the same frequency in males and females.
Jiri Homolka, No Financial Disclosure Information; No Product/Research Disclosure Information