PURPOSE: Although pulmonary rehabilitation is effective for improving exercise capacity in interstitial lung disease patients, especially those with reduced exercise capacity, the influence of multiple complications of systemic sclerosis on improvement is unknown. This study was performed to determine the factors affecting exercise outcome in patients with systemic sclerosis.
METHODS: Thirty outpatients (20 women, 10 men) with a mean age of 56 years, vital capacity of 77% predicted, carbon monoxide diffusing capacity of 45% predicted, and baseline six-minute walking distance of 459 meters (84% predicted) were examined regarding changes in six-minute walking distance at a mean interval of 52 days under medication controlled with corticosteroid. Eighteen subjects, chosen in chronological order, participated in an additional pulmonary rehabilitation program at home for a mean of 45 days during the study period. They had not been instructed regarding exercise as pulmonary rehabilitation before this study.
RESULTS: Six-minute walking distance improved significantly in patients with exercise but not in those without exercise (P<0.001, P=0.14, respectively). The change in distance was inversely correlated with baseline walking distance (R2=0.557) in patients with exercise, while there was no significant correlation in those without exercise (R2=0.001). The improvement in distance by exercise was not related to age, duration of disease, vital capacity, carbon monoxide diffusing capacity, modified Rodnan skin score, basal metabolic rate, or interstitial pneumonia markers (KL-6 and SP-D), but was weakly related to right ventricle systolic pressure (R2=0.240). A similar improvement was observed with or without cyclophosphamide pulse therapy. The basal metabolic rate in ten patients with exercise was slightly reduced but the effect was not significant.
CONCLUSIONS: The improvement in walking distance by exercise was more effective in systemic sclerosis patients with reduced exercise capacity and higher right ventricle systolic pressure, and was unaffected by pulmonary function, skin sclerosis, inflammation status of disease, and medication.
CLINICAL IMPLICATIONS: These findings demonstrated the usefulness of pulmonary rehabilitation for systemic sclerosis despite multiple complications.
DISCLOSURE: The following authors have nothing to disclose: Fujiko Someya, Naoki Mugii
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