Pulmonary Langerhans' Cell Histiocytosis and Diabetes Insipidus: On the occasion of two cases

Abstract

Pulmonary Langerhans' cell histiocytosis (PLCH) is an uncommon interstitial lung disease with an unknown etiology in smokers. Disease is usually seen with isolated lung involvement and central nervous system involvement is rare. Two PLCH cases, with central diabetes insipidus (DI) determined at diagnosis in one of the cases and during follow-up in the other, is being presented in this report. The patients' ages were 39 and 24, respectively and they were both male patients with a history of smoking. Widely cystic lesions were viewed at high-resolution computed tomography scans (HRCT) and PLCH was detected at the biopsies obtained by video-assisted thoracoscopic surgery (VATS), in both two cases. Therapy was started with 0.5 mg/kg methyl prednisolone for latter case, because of impairment in pulmonary function tests during follow up. On control, after one month therapy, partial improvement was seen for PLCH and the therapy was continued up to 6 months. At the end of the therapy complete remission was seen and corticotherapy was stopped. Desmopressin was started for both cases at 0.1 mg/day, orally. After one month of treatment clinical improvement was observed during control.