Predictors of poor outcomes in juvenile dermatomyositis: what do we know? A narrative review

Abstract

Objective. Juvenile dermatomyositis (JDM) is a rare chronic systemic inflammatory disorder with a highly variable clinical course. It is important to identify the patients at risk of developing more severe disease. However, based on the existing literature, there is a lack of data regarding predictors of poor outcomes. Obtaining knowledge about clinical and laboratory risk factors for disease progression and severity at an earlier stage of the disease could potentially lead to a better long-term prognosis for patients with JDM. Methods. A narrative review to identify risk factors for poor outcomes in patients with JDM, such as death, severe disease, refractory disease, and functional impairment, was conducted. A total of 27 articles were included. Results. Certain clinical manifestations and immunology features appear to worsen the prognosis in children with JDM. The recognition of these risk factors is essential for all pediatric rheumatologists as it allows the earlier identification of patients with potentially worse outcomes. These patients should receive closer follow-up and aggressive and individualized therapy to reduce their morbimortality. Conclusions. Additional research is needed not only to identify more predictors of worse outcomes but also more effective treatment approaches targeted toward these patients important to identify the patients at risk of developing a more severe disease. However, based on the existing literature, there is a lack of data regarding predictors of poor outcomes. Obtaining knowledge about clinical and laboratory risk factors for disease progression and severity at an earlier stage of the disease could potentially lead to a better long-term prognosis for patients with JDM. Therefore, to help physicians identify patients at risk of developing severe disease, we aim to review the literature and identify different outcomes (such as mortality, severe disease, refractory disease, and functional impairment) and possible associated risk factors.