Long-term clinical course and outcomes in patients with lymphangioleiomyomatosis

ABSTRACT:

Background

Lymphangioleiomyomatosis (LAM) is a rare multisystemic disorder with various clinical manifestations. Despite the recognition of several prognostic factors, the long-term clinical course and prognosis of patients with LAM in the era of sirolimus therapy are not established.

Methods

The clinical data of 104 patients with LAM were retrospectively analyzed. Death or lung transplantation was defined as the primary outcome. Disease progression (DP) was defined as a 10% absolute decline in forced expiratory volume in one second (FEV1).

Results

The mean age of all patients was 40.3 years. Over a median follow-up period of 7.1 years, of all patients, 6.7% died and 1.9% underwent lung transplantation, while of 92 patients with serial lung function data, 35.9% e

SUBMITTER: Yoon H 

PROVIDER: S-EPMC9206248 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature