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Original Research: Diffuse Lung Disease |

St. George’s Respiratory Questionnaire Has Longitudinal Construct Validity in LymphangioleiomyomatosisQuality of Life in Lymphangioleiomyomatosis

Jeffrey J. Swigris, DO, MS; Hye-Seung Lee, PhD; Marsha Cohen, MD; Yoshikazu Inoue, MD; Joel Moss, MD, PhD, FCCP; Lianne G. Singer, MD, FCCP; Lisa R. Young, MD; Francis X. McCormack, MD, FCCP; for the National Institutes of Health Rare Lung Diseases Consortium and the Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus Trial Group
Author and Funding Information

From the Autoimmune Lung Center and Interstitial Lung Disease Program (Dr Swigris), National Jewish Health, Denver, CO; the Pediatrics Epidemiology Center (Dr Lee), University of South Florida College of Medicine, Tampa, FL; the Women’s Health Research Institute (Dr Cohen), Women’s College Hospital, Toronto, ON, Canada; the National Hospital Organization Kinki-Chou Chest Medical Center (Dr Inoue), Osaka, Japan; the National Institutes of Health (Dr Moss), Bethesda, MD; the Lung Transplantation Program (Dr Singer), University of Toronto; the Division of Allergy, Pulmonary, and Critical Care Medicine (Dr Young), Vanderbilt University School of Medicine, Nashville, TN; and the Department of Internal Medicine (Dr McCormack), Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, Cincinnati, OH.

Correspondence to: Jeffrey J. Swigris, DO, MS, Associate Professor of Medicine, Autoimmune Lung Center and Interstitial Lung Disease Program, National Jewish Health, Southside Bldg, Office #G011, 1400 Jackson St, Denver, CO 80206; e-mail: swigrisj@njc.org


Funding/Support: Dr Swigris is supported in part by a Career Development Award from the National Institutes of Health (NIH) [Grant K23 HL092227]. Dr Moss was supported by the Intramural Research Program, NIH, National Heart, Lung and Blood Institute. The Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus Trial was supported by the NIH Office of Rare Disease Research [Grant RR019498]; the US Food and Drug Administration [Grant FD003362]; the Japanese Ministry of Health, Labour, and Welfare; the Canadian Institutes of Health Research; Pfizer Pharmaceuticals; The LAM Foundation; the Tuberous Sclerosis Alliance; and Cincinnati Children’s Hospital.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2013;143(6):1671-1678. doi:10.1378/chest.12-0161
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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:  SGRQ scores correlated with the majority of anchor values at baseline, 6 months, and 12 months. Results from longitudinal analyses demonstrated that SGRQ change scores tracked changes over time in values for each of the four anchors. At 12 months, subjects with the greatest improvement from baseline in FEV1 experienced the greatest improvement in SGRQ scores (Symptoms domain, −13.4 ± 14.6 points; Activity domain, −6.46 ± 8.20 points; Impacts domain, −6.25 ± 12.8 points; SGRQ total, −7.53 ± 10.0 points). Plots of cumulative distribution functions further supported the longitudinal validity of the SGRQ in LAM.

Conclusions:  In LAM, SGRQ scores are associated with variables used to assess LAM severity. The SGRQ is sensitive to change in LAM severity, particularly when change is defined by FEV1, perhaps the most clinically relevant and prognostically important variable in LAM. The constellation of results here supports the validity of the SGRQ as capable of assessing longitudinal change in HRQL in LAM.

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