RESUMEN
Lymphangioleiomyomatosis (LAM) is a rare lung disease of unknown etiology, described since 1918 associated with tuberous sclerosis complex (TSC-LAM) and are reported sporadically (S-LAM). It is classified within the group of interstitial lung diseases (ILD) and currently the European Respiratory Society (ERS) has published guidelines for diagnosis and treatment. The objective is to evaluate the clinical presentation of two patients, evolution, management, and review of current treatment. Both patients admitted in our hospital for examination, presenting with spontaneous pneumothorax. Their CT scan shows characteristic cystic lesions and thoracotomy with lung biopsy was performed because lack of expansion and to confirming the diagnosis. Despite the controversy about the optimal management of these patients, there is evidence about the use of progesterone routinely in patients with rapid deterioration of respiratory function when it was provided for a period of at least 12 months. Due to the rareness of the disease, it requires a patient registry to evaluate the use of experimental drugs or include them in research protocols to improve their prognosis.