Pathophysiology and Therapy of Cardiac Dysfunction in Duchenne Muscular Dystrophy

Daniel P. Judge; David A. Kass; W. Reid Thompson; Kathryn R. Wagner


Am J Cardiovasc Drugs. 2011;11(5):287-294. 

In This Article

5. Glucocorticoids

Muscle inflammation is a poorly understood contributor to DMD. Glucocorticoid treatment prolongs ambulation by 1–2 years in patients with the disorder, likely due to the antiinflammatory effects of the glucocorticoids.[26] With this in mind, a retrospective analysis of cardiac function in individuals withDMDand normal cardiac function compared patients who received glucocorticoid treatment (n = 14) with those who did not (n = 23), with an average length of follow-up of >4 years.[27] At the final evaluation, 16 untreated versus two treated cases had evidence of ventricular dysfunction (p < 0.001).[27] Despite concerns that glucocorticoids may raise BP, increase BMI, and contribute to both gonadal dysfunction and osteopenia, beneficial effects likely outweigh difficulties. This trial and the improved longevity for individuals with DMD that is associated with increased use of glucocorticoids in many Western countries support the impression that glucocorticoids have a protective effect in DMD that does not exclude the heart.[7]


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