• Miharu Kato, Yumiko Kanehiro, Kaku Yoshimi, Yuzo Kodama, Mitsuaki Sekiya, Tadashi Sato, Kazuhisa Takahashi, Kuniaki Seyama, and Multicenter Lymphangioleiomyomatosis Sirolimus Trial for Safety Study Group..
• Divisions of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-Ku, Tokyo 113-8431, Japan. Electronic address: mkato@juntendo.ac.jp.
• Respir Investig. 2018 Nov 1; 56 (6): 480-488.

BackgroundLymphangioleiomyomatosis (LAM) is a progressive, cystic lung disease that causes an obstructive ventilatory impairment similar to chronic obstructive pulmonary disease (COPD) and impairs the health-related quality of life (HRQoL). Here, we extended the use of the COPD assessment test (CAT) to patients with chronic respiratory diseases other than COPD. Specifically, the CAT was administered to patients with LAM for the first time.MethodsUsing data from 25 patients with LAM at Juntendo University who participated in the Multicenter Lymphangioleiomyomatosis Sirolimus Trial for Safety (MLSTS), we evaluated changes in pulmonary function, responses to HRQoL questionnaires (the CAT, St. George׳s Respiratory Questionnaire [SGRQ], EuroQOL Visual Analogue Scale [EuroQOL-VAS], and Functional Performance Inventory [FPI]), and the association between pulmonary function and HRQoL during a 24-month period of sirolimus treatment.ResultsForced expiratory volume in one second (FEV1), forced vital capacity (FVC), and CAT total scores improved over the 24 months of sirolimus treatment (5.33 ± 1.20 ml/month, 2.61 ± 1.16 ml/month, and -0.127 ± 0.022 score/month, respectively), whereas SGRQ total score, EuroQOL-VAS score, and FPI score did not. Most pulmonary functions at baseline were associated with the CAT breathlessness score during the first year. Longitudinal changes in FEV1, FEV1%predicted, or FEV1/FVC correlated significantly with the scores of CAT total, CAT breathlessness, and SGRQ activity. When analyzed by stepwise multivariate regression within a linear mixed-effects model, CAT breathlessness and confidence scores were significantly associated with a change in FEV1 from the baseline value (P = 0.0011, and P = 0.0441).ConclusionOur results suggest that the CAT is a useful instrument for assessing HRQoL in sirolimus-treated patients with LAM.Copyright © 2018 The Japanese Respiratory Society. Published by Elsevier B.V. All rights reserved.

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