Clinical experience of lymphangioleiomyomatosis in the UK

Background-Lymphangioleiomyomatosis is a rare lung disease that affects onl y women. No controlled trials of management have been performed and, until such data are available, management must be based on clinical experience. T his study provides data on the natural history of lymphangioleiomyomatosis in the UK and compares this with experience from other centres. Methods-We tried to identify all cases of lymphangioleiomyomatosis in the U K over a five year period by contacting all chest physicians. Cases were co nfirmed by lung biopsy or history and high resolution computed tomographic (CT) scanning. Details of disease and management were obtained from hospita l notes. Results-The 50 patients who fitted the diagnostic criteria for lymphangiole iomyomatosis had a median age at onset of 35 years (range 52-50). Five pres ented when postmenopausal (four taking hormone replacement therapy). Pneumo thorax and dyspnoea were the most common presenting features. Extrapulmonar y presentations included renal angiomyolipomas (3) and lymphangiomyomas (2) . Only half the patients were assessed for renal angiomyolipoma and six wer e identified. Thirty patients had had one or more pneumothoraces, of which two thirds recurred if treated conservatively. Chylous effusions occurred i n 11 patients, five requiring surgery. Pregnancy was uncommon once the diag nosis was made (n=7), but was associated with an increase in complications. Half the patients were taking a beta agonist and many showed a bronchodila tor response in the laboratory. Thirty six patients had received hormone tr eatment. Conclusions-Our UK five year period prevalence was one per 1.1 million popu lation. Since prophylactic interventions are sometimes indicated for renal angiomyolipoma, these data suggest that screening for angiomyolipoma, ideal ly by CT scanning, may be underused. Patients need to be aware of the incre ase in complications associated with pregnancy. Recurrence rate of pneumoth orax was high in those not treated surgically. Hormone treatment was used v ariably and controlled trials are needed to determine their role and the op timum duration and dose.