European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis.

We have generated the first international clinical guidelines for LAM. As LAM is a multisystem rare disease, this required the participation of a range of specialists from many countries, particularly Europe and the USA. The absence of a strong evidence base for this rare disease required the use of a consensus technique to provide guidelines containing the best synthesis of evidence and expert opinion. These guidelines contain the first proposal of diagnostic criteria in LAM and recommend investigations and work-up for patients, screening of groups at risk for LAM, discussion of prognosis and management. The overall aim of this document is to help patients by achieving an earlier diagnosis and better care, including in countries with differing levels of healthcare resources. The guidelines will be reviewed regularly or in real time should a major advance occur.