St. George's Respiratory Questionnaire has longitudinal construct validity in lymphangioleiomyomatosis.

ABSTRACT:

Background

Lymphangioleiomyomatosis (LAM) is an uncommon, progressive, cystic lung disease that causes shortness of breath, hypoxemia, and impaired health-related quality of life (HRQL). Whether St. George's Respiratory Questionnaire (SGRQ), a respiratory-specific HRQL instrument, captures longitudinal changes in HRQL in patients with LAM is unknown.

Methods

Using data from the Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus trial, we performed analyses to examine associations between SGRQ scores and values for four external measures (anchors). Anchors included (1) FEV₁, (2) diffusing capacity of the lung for carbon monoxide, (3) distance walked during the 6-min walk test, and (4) serum vascular endothelial growth factor-D.

Results

SUBMITTER: Swigris JJ 

PROVIDER: S-EPMC3673663 | biostudies-literature | 2013 Jun

REPOSITORIES: biostudies-literature