A 4-year prospective evaluation of protocols to improve clinical outcomes for patients with lymphangioleiomyomatosis in a national clinical centre.

Lymphangioleiomyomatosis (LAM) is a rare multisystem disease. Progressive airflow limitation, pneumothorax and angiomyolipoma-related bleeding are major morbidities. As treatments are available for these complications, we prospectively audited loss of FEV1 (ΔFEV1), pneumothorax and angiomyolipoma bleeding against clinical standards over 4 years at the UK Clinical Centre. ΔFEV1 for these patients is lower than previously reported and rates of pneumothorax and angiomyolipoma haemorrhage are low. This suggests that real-time analysis of clinical data with targeted interventions can reduce morbidity in LAM. These measures could be applied as quality standards to compare the emerging LAM clinical networks worldwide. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/