1. COPD assessment test as a possible tool for evaluating health-related quality of life in lymphangioleiomyomatosis
- Author
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Kazuhisa Takahashi, Kuniaki Seyama, Yumiko Kanehiro, Mitsuaki Sekiya, Kaku Yoshimi, Tadashi Sato, Yuzo Kodama, and Miharu Kato
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Adult ,Male ,Pulmonary and Respiratory Medicine ,Vital capacity ,medicine.medical_specialty ,Lung Neoplasms ,Time Factors ,Visual analogue scale ,Pulmonary function testing ,Pulmonary Disease, Chronic Obstructive ,03 medical and health sciences ,FEV1/FVC ratio ,0302 clinical medicine ,Quality of life ,Forced Expiratory Volume ,Surveys and Questionnaires ,Internal medicine ,medicine ,Humans ,Lung volumes ,Lymphangioleiomyomatosis ,030212 general & internal medicine ,Sirolimus ,COPD ,Antibiotics, Antineoplastic ,business.industry ,Middle Aged ,medicine.disease ,Respiratory Function Tests ,respiratory tract diseases ,030228 respiratory system ,Multivariate Analysis ,Quality of Life ,Regression Analysis ,Female ,business - Abstract
Background Lymphangioleiomyomatosis (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. Methods Using 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. Results Forced expiratory volume in one second (FEV 1 ), 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 FEV 1 , FEV 1% predicted, or FEV 1 /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 FEV 1 from the baseline value ( P = 0.0011, and P = 0.0441). Conclusion Our results suggest that the CAT is a useful instrument for assessing HRQoL in sirolimus-treated patients with LAM.
- Published
- 2018
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