Juvenile Dermatomyositis

Lauren M. Pachman; Sarah Tansley; Ann M. Reed; Clarissa M. Pilkington; Brian M. Feldman; Lisa G. Rider

doi:10.1007/978-3-031-23488-0_17

Juvenile Dermatomyositis

Lauren M. Pachman, Sarah Tansley, Ann M. Reed, Clarissa M. Pilkington, Brian M. Feldman, Lisa G. Rider

Research output: Chapter or section in a book/report/conference proceeding › Chapter or section

Abstract

• Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy (IIM) of childhood. Although JDM shares some risk factors, clinical and laboratory features with its adult counterpart, a number of features distinguish JDM from adult DM. • The incidence of JDM is approximately 3.2 cases/million children/year with a gender ratio of 2 girls/1 boy. • The mean age at diagnosis of JDM is approximately 7 years of age, but more than 25% of patients are 4 years old or younger. • The duration of active, untreated disease before diagnosis strongly influences patients’ features at presentation and clinical outcomes. • The occurrence of malignancy in association with JDM is extremely uncommon. This contrasts significantly with adult DM, in which paraneoplastic disease is common. In adult DM, anti-TIF-1γ and anti-NXP2 (MJ) autoantibodies often signal the presence of a concomitant malignancy. • Environmental and genetic factors, such as the composition of their HLA alleles, impact both the child’s susceptibility to myositis and the inflammatory response, adding heterogeneity to disease pathophysiology.

Original language	English
Title of host publication	A Clinician's Pearls and Myths in Rheumatology (2nd ed.)
Editors	John H. Stone
Place of Publication	Cham, Switzerland
Publisher	Springer International Publishing
Pages	275-285
Number of pages	11
ISBN (Electronic)	9783031234880
ISBN (Print)	9783031234873
DOIs	https://doi.org/10.1007/978-3-031-23488-0_17
Publication status	Published - 31 Mar 2023

Keywords

Calcinosis
Dermatomyositis
Glucocorticoids
Gottron’s papules
Heliotrope rash
Juvenile myositis
Lipodystrophy
Myositis-specific antibody
Myositis-specific autoantibodies
Perifascicular atrophy
Periungual nailfold capillary changes

ASJC Scopus subject areas

General Medicine
General Biochemistry,Genetics and Molecular Biology

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10.1007/978-3-031-23488-0_17

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title = "Juvenile Dermatomyositis",

abstract = "• Juvenile dermatomyositis (JDM) is the most common idiopathic inflammatory myopathy (IIM) of childhood. Although JDM shares some risk factors, clinical and laboratory features with its adult counterpart, a number of features distinguish JDM from adult DM. • The incidence of JDM is approximately 3.2 cases/million children/year with a gender ratio of 2 girls/1 boy. • The mean age at diagnosis of JDM is approximately 7 years of age, but more than 25% of patients are 4 years old or younger. • The duration of active, untreated disease before diagnosis strongly influences patients{\textquoteright} features at presentation and clinical outcomes. • The occurrence of malignancy in association with JDM is extremely uncommon. This contrasts significantly with adult DM, in which paraneoplastic disease is common. In adult DM, anti-TIF-1γ and anti-NXP2 (MJ) autoantibodies often signal the presence of a concomitant malignancy. • Environmental and genetic factors, such as the composition of their HLA alleles, impact both the child{\textquoteright}s susceptibility to myositis and the inflammatory response, adding heterogeneity to disease pathophysiology.",

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Juvenile Dermatomyositis

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Keywords

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