Harari S, Torre O, Elia D, et al. - Following the publication of the ERS and American Thoracic Society/Japanese Respiratory Society guidelines and the introduction of sirolimus, researchers examined the changes in the management and the natural history of lymphangioleiomyomatosis (LAM), which is a rare disease. They analyzed 162 LAM patients, focusing on clinical features and diagnostic approach. A histological diagnosis was made in 61 patients (22 from 2010 onward). A radiological diagnosis was received in 101 patients based on the guidelines criteria. Post-10 years, a cumulative incidence of 25.5% was estimated for mortality in the whole population. In patients who entered the study since 2011 (after publication of the MILES trial), a significantly lower cumulative incidence of mortality was observed after 5 years, vs patients who entered the study before. In this large cohort of patients with functional impairment and other manifestations of the disease, long-term efficacy of sirolimus therapy was evident. As per data, the natural history of the disease is altered, leading to reduction in mortality as well as the requirement of invasive diagnostic techniques, as a result of the advent of sirolimus and the publication of international guidelines.