Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy in childhood. The most common findings are muscle weakness and erythematous rash. Less frequently, the lungs, gastrointestinal system (GIS), joints, and other organs are affected [1]. Corticosteroids and methotrexate are used in treatment. Complications such as calcinosis, lipodystrophy, interstitial lung disease, and joint contractures are difficult to manage [2]. Disease-modifying anti-rheumatic drugs (DMARDs) other than methotrexate, biologics, and small molecules are used in resistant cases [2].

Interferon (IFN) stimulated or regulated genes are upregulated in JDM, similar to systemic lupus erythematosus [3]. IFNs activate the Janus kinase (JAK)-signal transducer and transcription (STAT) pathway, which leads to the transcription of IFN-stimulated genes. This pathway is the target for the blockade of the transcription of IFN genes. Tofacitinib inhibits JAK1/3, while ruxolitinib and baricitinib inhibit JAK 1/2 [4]. Clinical trials regarding to use of specific JAK inhibitors in JDM are ongoing [5,6]. However, the use of JAK inhibitors in JDM is mostly limited to tofacitinib, ruxolitinib, and baricitinib.

In this review, we aimed to perform a detailed analysis of the published data regarding the use of JAK inhibitors in JDM treatment.