Pulmonary Langerhans' cell granulomatosis - (Histiocytosis X): Clinical analysis of 8 cases

A detailed retrospective analysis of 8 cases has been set up to obtain an u pdate of the clinical, radiological, diagnostic and therapeutical aspects o f pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histi ocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD ) in a registration by pneumologists in Flanders. Seventy five % of our pat ients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax d uring the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. R adiologically nodules and/or cystic lesions were found with preserved volum es. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchi al biopsies (TBB) were found to be not sensitive. Systemic staging in our g roup showed 50% of the patients to have a second focus of organ involvement . Treatment with corticosteroids and possibly immunosuppressives was withou t clear effect. Since 25% of our patients ultimately became candidate for l ung transplantation extrapulmonary staging is prerogative and the high freq uency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contrain dication for lung transplantation in some but not all institutions.