Cystic lung disease lymphangioleiomyomatosis (LAM) in a male patient

Lymphangioleiomyomatosis (LAM) is characterised by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes and other organs. We report the case of a man in his 50s who had right-sided pleural effusion. On performing a diagnostic tap, the fluid was milky white in colour. An intercostal chest tube was inserted and after complete drainage of fluid, a high-resolution CT (HRCT) was done. HRCT revealed multiple cysts throughout both of the lungs. On subsequent bronchoscopy-guided transbronchial lung biopsy and histochemical staining, diagnosis of LAM was made. We started the patient on oral sirolimus. On subsequent follow-up, subjective and objective improvements were observed.