UNASSIGNED: @entity8817 ( @entity8817 ) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common @entity5442 of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of @entity1 with @entity8817 at a referral pediatric rheumatology center in @entity233 . We retrospectively reviewed medical records of @entity1 diagnosed with @entity8817 between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty. A total of 50 @entity1 (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 @entity1910 (100%), Gottron papule (96%), @entity425 (90%), @entity5658 (88%), and @entity730 (38%). All @entity1 had elevated muscle enzymes at the disease onset. Sixty-eight percent of the patients had anti-nuclear antibody positivity. Electromyography on 27 @entity1 and muscle biopsy on 14 @entity1 were performed, and all of them showed signs of @entity8817 . Early aggressive treatment with @entity288 mostly in combination with @entity1499 was used. @entity205 was added to 48% of the @entity1 ' treatment regimen in case of severe or refractory disease. All @entity1 except two cases, who were referred to our clinic after long disease duration with widespread @entity730 , achieved remission. Early diagnosis and early initiation of intensive therapy are important in reducing @entity8817 complications. International collaboration is needed in order to better understanding and management of the disease.

XXXX : a tertiary center experience.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity5442 :: ('MESH:D009220', 'Disease') :: ['idiopathic inflammatory myopathy'] @entity233 :: ('9103', 'Species') :: ['Turkey'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['heliotrope rash'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity8817 :: ('MESH:C538250', 'Disease') :: ['juvenile dermatomyositis', 'JDM', 'Juvenile dermatomyositis'] @entity730 :: ('MESH:D002114', 'Disease') :: ['calcinosis'] @entity205 :: ('MESH:D016572', 'Chemical') :: ['Cyclosporine'] @entity5658 :: ('MESH:D003873', 'Disease') :: ['erythroderma'] @entity1499 :: ('MESH:D008727', 'Chemical') :: ['methotrexate'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity8817 :: (MESH:C538250,Disease) :: ['Juvenile dermatomyositis']
