A New Outlook on Clinical Course of Juvenile Dermatomyositis - Experience of a Single Center

Abstract

mild muscle symptoms that are easy to treat and never relapse, to a therapy-resistant chronic condition [5].JDM diagnosis is based on the Bohan & Peter criteria [6] originally developed for adult patients: symmetric proximal muscle weakness, biopsy-proven myositis, elevated serum muscle enzyme levels, electromyographic changes of myositis. JDM may be also misdiagnosed as polymyositis in patients presenting with isolated muscle symptoms on first admission [7].The pathogenesis of JDM is not completely understandable yet. Viral infection or immune dysfunction may trigger disease in patients with genetic predispositions [1]. Accordingly, the identification of novel autoantibodies in JDM (such as anti-p155/140, anti-p140) may have clinical implications, as they are associated with specific clinical features, treatment response and prognosis [8].Dermatomyositis is also considered as one of paraneoplastic syndromes in adults. It is believed to occur in 7-15% of all cancer patients [9]. The association of dermatomyositis and cancer is more likely to happen in elderly people [10], but it may also coincide with leukemia and lymphoma in pediatric patients [11].As JDM differs from the course of disease in adults, diagnostic criteria require a new approach. They do not consider radiologic methods such as MRI, which is recently becoming the preferred non-invasive test indicating muscle inflammation, instead of muscle biopsy and electromyography (EMG) included in the criteria [3].In order to adjust criteria to pediatric patients, differences in clinical course of dermatomyositis between adults and children need to be reported. Therefore we present series of 5 cases. Materials and MethodsThe retrospective study included the medical charts of 8 patients ≤18 years of age who were treated due to JDM suspicion at the