Am. J. Respir. Crit. Care Med., Volume 164, Number 12, December 2001, 2191-2194

Changes in Spirometry Over Time as a Prognostic Marker in Patients with Duchenne Muscular Dystrophy

MARGARET F. PHILLIPS, ROSALINE C. M. QUINLIVAN, RICHARD H. T. EDWARDS, and PETER M. A. CALVERLEY

Pulmonary and Rehabilitation Research Group, University Hospital Aintree, Fazakerley, Liverpool; Muscle Clinic, Robert Jones and Agnes Hunt Orthopaedic and District Hospital, Oswestry, Shropshire, United Kingdom

Duchenne muscular dystrophy (DMD) causes a progressive impairment of muscle function leading to hypercapnic respiratory failure. Most studies of respiratory function in DMD have been cross-sectional rather than longitudinal, and these data have not been related to survival. We retrospectively studied 58 patients with DMD with at least 2 yr of follow-up spirometry and known vital status. Spirometry was abnormal at entry: median FEV₁ 1.60 L (range 0.4 to 2.6 L), FVC 1.65 L (range 0.45 to 2.75 L), FVC 64% predicted (range 29 to 97%). Individual rates of change of vital capacity varied, with a median annual change of 0.18 L (range 0.04 to 0.74 L), 8.0% predicted FVC (range 2 to 39%). During the study 37 patients died; the median age of death, calculated by Kaplan-Meier analysis, was 21.5 yr (range 15 to 28.5 yr). The age when vital capacity fell below 1 L was a strong marker of subsequent mortality (5-yr survival 8%). The maximal vital capacity recorded and its rate of decline (however expressed) predicted survival time. Repeated spirometric measurement provides a simple and relatively powerful means of assessing disease progression in these patients and should be considered when planning treatment trials.

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