A juvenile dermatomyositis: demographics, characteristics and disease outcome in an Egyptian cohort.

Abstract

To study the demographics, characteristics, management and disease outcome of Egyptian children with juvenile dermatomyositis (JDM). Retrospective analysis of the records of 134 JDM patients attending two centres in Cairo, Egypt from January 2010 to December 2019. A total of 128 patients were included in the study, all of which fulfilled either the Bohan and Peter criteria and/or the EULAR/ACR classification criteria of 2017. The mean age of disease onset was 5.9±2.8 years and the follow-up duration were 6±3.2 years. Female to male ratio was 2.2:1. Constitutional manifestations and cutaneous skin ulcers were common, while gut vasculopathy was rare in our patients. Heliotrope rash was the commonest skin manifestation. Lactate dehydrogenase enzyme was more frequently elevated than creatine kinase. Electromyography was the most frequently used diagnostic procedure, while muscle biopsy and muscle MRI were not commonly done in our patients. Glucocorticoids, methotrexate, hydroxychloroquine, mycophenolate mofetil and IVIG were the most frequently used medications. Sixty (46.9 %) of the patients had clinically inactive disease, at the last follow-up visit. Chronic skin disease, residual muscle weakness, calcinosis and growth failure were among the most common cumulative damage manifestations. The mortality rate was 1.6% over the follow-up period, one death was due to severe infection, and the other due to respiratory failure. Although our patients shared several similarities with their peers in the Middle East and in Europe, there were some striking differences. These differences can be attributed to the ethnic and environmental disparities.