CHEST Guidelines-The-Impact-of-Corticosteroids-on-Cardiopulmonary-P

The-Impact-of-Corticosteroids-on-Cardiopulmonary-P

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The editorial discusses the impact of corticosteroids on cardiopulmonary progression in patients with Duchenne Muscular Dystrophy (DMD), a genetic disorder marked by progressive loss of muscle function. Cardiopulmonary failure is the primary cause of mortality in DMD. For over 30 years, corticosteroids have been the main disease-modifying treatment, proven to preserve motor function and delay pulmonary decline.<br /><br />A study by Trucco et al., conducted at the Dubowitz Neuromuscular Center, analyzed 293 patients from 2000 to 2017. The research focused on the effects of different corticosteroid treatments—prednisone and deflazacort, administered either daily or intermittently—on pulmonary and cardiac status. Findings revealed that corticosteroid-naïve patients experienced a faster decline in forced vital capacity (FVC % predicted) and required nocturnal ventilation approximately three years earlier than those treated with corticosteroids. Those on daily corticosteroids attained higher FVC % predicted values than those on an intermittent schedule, though shorter stature was noted as a side effect.<br /><br />Cardiac evaluations showed that corticosteroid-naïve patients had a higher prevalence of cardiomyopathy and developed it at a younger age compared to those undergoing treatment. The editorial underlines the positive impact of corticosteroids on respiratory health and myocardial function in DMD patients. However, while pulmonary function differed between corticosteroid regimens, myocardial progression did not show significant variance.<br /><br />This editorial underscores the importance of corticosteroids in managing DMD, providing insight into different treatment regimens. Despite potential side effects, such as shorter stature, corticosteroids notably contribute to improved respiratory and cardiac outcomes in DMD patients, emphasizing their critical role in therapy. The study adds depth to the understanding of corticosteroid usage in DMD care, noting distinctions in regimen effects on pulmonary function and myocardial dysfunction.

Keywords

Duchenne Muscular Dystrophy

corticosteroids

cardiopulmonary progression

prednisone

deflazacort

forced vital capacity

cardiomyopathy

respiratory health

myocardial function

treatment regimens