Juvenile dermatomyositis: a tertiary center experience

Abstract

Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty. A total of 50 patients (35 females), median age at the onset 6.1 +/- 4.1 years, were identified. Mean follow-up period was 74.5 +/- 49.7 months. Presenting clinical symptoms included heliotrope rash (100%), Gottron papule (96%), muscle weakness (90%), erythroderma (88%), and calcinosis (38%). All patients had elevated muscle enzymes at the disease onset. Sixty-eight percent of the patients had anti-nuclear antibody positivity. Electromyography on 27 patients and muscle biopsy on 14 patients were performed, and all of them showed signs of juvenile dermatomyositis. Early aggressive treatment with corticosteroids mostly in combination with methotrexate was used. Cyclosporine was added to 48% of the patients' treatment regimen in case of severe or refractory disease. All patients except two cases, who were referred to our clinic after long disease duration with widespread calcinosis, achieved remission. Early diagnosis and early initiation of intensive therapy are important in reducing JDM complications. International collaboration is needed in order to better understanding and management of the disease.