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

Abstract

Introduction: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in children. Diagnostic criteria by Bohan & Peter are originally formulated for adults. A wide range of differences in clinical course of dermatomyositis between adults and children has provoked to set a new look at the existing criteria. Objective: The aim of our study was to analyze the clinical course and reaction to administered drug therapy in association with laboratory and electromyographic findings in pediatric patients with dermatomyositis. Materials and methods: The retrospective analysis included 5 children with JDM hospitalized in Department of Pediatric Cardiology and Rheumatology Medical University of Lodz, Poland between October 2000 and November 2014. Demographic data, characteristics of clinical symptoms, laboratory tests, EMG and muscle biopsy reports and reaction to administered treatment were analyzed. Results: Muscle weakness was the most common symptom observed in 80% of patients. Other symptoms present in more than a half of patients included heliotrope rash (60%) and Gottron sign (60%). Muscle enzyme levels were significantly elevated in 4 out of 5 patients. Electromyography (EMG) was performed in 3 patients and was positive in every case, as well as muscle biopsy. Initial therapy involved prednisone 1mg/kg b.w./day in all patients combined with disease modifying anti-rheumatic drug, whether methotrexate was the most common choice (60%). Complications which occurred in presented patients involve: rhabdomyolysis, severe calcinosis and contractures of elbows, exacerbation after reduction of GCS dose, hypertriglyceridemia, steatohepatitis, tachycardia.