The use of corticosteroids in patients with Duchenne muscular dystrophy (DMD) after their loss of ambulation (LOA) may delay the onset of abnormal percent predicted forced vital capacity (ppFVC), according to an article published in Muscle & Nerve.

Boys with DMD who continued corticosteroid use for at least 1 year after LOA had a statistically older mean age at onset of ppFVC compared to boys who discontinued use within 1 year of LOA (P =.01) or those who were never treated with them (P =.04). No significant difference in time between LOA and onset of abnormal ppFVC was observed, however.

Continuation of corticosteroids for more than 1 year after LOA led to a significantly shorter time from LOA to abnormal left ventricular function compared to boys who didn’t have corticosteroids (P =.047) or who stopped taking them within a year of their LOA (P =.047). The average age at the first abnormal left ventricular function was not different between the 3 groups.


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“Our findings highlight the complexity of treatment with corticosteroids for DMD and the importance of additional studies to determine the benefits and risks of prolonged treatment,” the authors said.

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The study also found that boys who continued taking corticosteroids walked longer than patients who did not receive corticosteroids or stopped treatment within a year of LOA.

“Prospective studies of corticosteroid use in boys with DMD who have lost ambulation may identify benefits and can better elucidate risks, allowing for more effective counseling of patients on continuing treatment after LOA,” the authors suggested.

After exclusions, a total of 398 nonambulatory patients with DMD were included in the analyses. Patients were recruited using the Muscular Dystrophy Surveillance, Tracking, and Research Network collaboration.

Of these patients, 101 had started corticosteroid treatment before LOA and continued it for 1 year or more after. Corticosteroids were never used by 193 of the patients, and 104 patients discontinued treatment within 1 year before or after LOA.

Reference

Butterfield RJ, Krikov S, Conway KM, et al. Evaluation of effects of continued corticosteroid treatment on cardiac and pulmonary function in non-ambulatory males with Duchenne muscular dystrophy from MD STARnet. Muscle Nerve. Published online January 7, 2022. doi:10.1002/mus.27490