Recent advances in juvenile idiopathic inflammatory myopathies

Floranne C. Ernste, Ann M. Reed

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Purpose of review: Ongoing research continues to advance our understanding of the juvenile idiopathic inflammatory myopathies (JIIMs). We review the recent contributions from the published literature about the classification, pathogenesis, assessment, and treatment of JIIMs in basic and translational science and clinical research in 2013 through early 2014. Recent findings: Large registries, such as the Childhood Arthritis and Rheumatology Research Alliance registry, are conducting trials to enhance our understanding of JIIMs. Ultraviolet radiation exposure 1 month prior to juvenile dermatomyositis (JDM) may trigger the onset of disease. Myositis-specific autoantibodies define clinical phenotypes in JIIMs. MRI is useful in diagnosing JDM and may be used as a disease assessment tool. Type 1 interferon genes and proteins are increasing in use as disease assessment tools, but larger, prospective, validation studies are needed. Moderate-to-intense physical activity is effective in increasing the aerobic capacity of JDM patients in remission. New criteria developed by the Paediatric Rheumatology International Trials Organization for classifying inactive disease in JDM have practical applicability to the current clinical practice and clinical trials as even after 16.8 years of symptom onset, over half of JDM patients still have active disease. Summary: There has been significant progress in understanding the clinical characteristics, diagnostic workup, treatment, disease assessment, and prognosis of JIIM patients, but more prospective treatment trials are needed, especially in light of the paucity of the current biologic treatment agents available.

Original languageEnglish (US)
Pages (from-to)671-678
Number of pages8
JournalCurrent Opinion in Rheumatology
Volume26
Issue number6
DOIs
StatePublished - 2014

Fingerprint

Myositis
Rheumatology
Registries
Research
Interferon Type I
Validation Studies
Biological Factors
Therapeutics
Autoantibodies
Arthritis
Juvenile dermatomyositis
Clinical Trials
Prospective Studies
Exercise
Pediatrics
Phenotype
Proteins

Keywords

  • Clinical characteristics
  • Immunopathogenesis
  • Juvenile dermatomyositis
  • Prognosis
  • Therapy

ASJC Scopus subject areas

  • Rheumatology

Cite this

Recent advances in juvenile idiopathic inflammatory myopathies. / Ernste, Floranne C.; Reed, Ann M.

In: Current Opinion in Rheumatology, Vol. 26, No. 6, 2014, p. 671-678.

Research output: Contribution to journalArticle

Ernste, Floranne C. ; Reed, Ann M. / Recent advances in juvenile idiopathic inflammatory myopathies. In: Current Opinion in Rheumatology. 2014 ; Vol. 26, No. 6. pp. 671-678.
@article{5e55806f4d1b433d897aef6beb24ddab,
title = "Recent advances in juvenile idiopathic inflammatory myopathies",
abstract = "Purpose of review: Ongoing research continues to advance our understanding of the juvenile idiopathic inflammatory myopathies (JIIMs). We review the recent contributions from the published literature about the classification, pathogenesis, assessment, and treatment of JIIMs in basic and translational science and clinical research in 2013 through early 2014. Recent findings: Large registries, such as the Childhood Arthritis and Rheumatology Research Alliance registry, are conducting trials to enhance our understanding of JIIMs. Ultraviolet radiation exposure 1 month prior to juvenile dermatomyositis (JDM) may trigger the onset of disease. Myositis-specific autoantibodies define clinical phenotypes in JIIMs. MRI is useful in diagnosing JDM and may be used as a disease assessment tool. Type 1 interferon genes and proteins are increasing in use as disease assessment tools, but larger, prospective, validation studies are needed. Moderate-to-intense physical activity is effective in increasing the aerobic capacity of JDM patients in remission. New criteria developed by the Paediatric Rheumatology International Trials Organization for classifying inactive disease in JDM have practical applicability to the current clinical practice and clinical trials as even after 16.8 years of symptom onset, over half of JDM patients still have active disease. Summary: There has been significant progress in understanding the clinical characteristics, diagnostic workup, treatment, disease assessment, and prognosis of JIIM patients, but more prospective treatment trials are needed, especially in light of the paucity of the current biologic treatment agents available.",
keywords = "Clinical characteristics, Immunopathogenesis, Juvenile dermatomyositis, Prognosis, Therapy",
author = "Ernste, {Floranne C.} and Reed, {Ann M.}",
year = "2014",
doi = "10.1097/BOR.0000000000000103",
language = "English (US)",
volume = "26",
pages = "671--678",
journal = "Current Opinion in Rheumatology",
issn = "1040-8711",
publisher = "Lippincott Williams and Wilkins",
number = "6",

}

TY - JOUR

T1 - Recent advances in juvenile idiopathic inflammatory myopathies

AU - Ernste, Floranne C.

AU - Reed, Ann M.

PY - 2014

Y1 - 2014

N2 - Purpose of review: Ongoing research continues to advance our understanding of the juvenile idiopathic inflammatory myopathies (JIIMs). We review the recent contributions from the published literature about the classification, pathogenesis, assessment, and treatment of JIIMs in basic and translational science and clinical research in 2013 through early 2014. Recent findings: Large registries, such as the Childhood Arthritis and Rheumatology Research Alliance registry, are conducting trials to enhance our understanding of JIIMs. Ultraviolet radiation exposure 1 month prior to juvenile dermatomyositis (JDM) may trigger the onset of disease. Myositis-specific autoantibodies define clinical phenotypes in JIIMs. MRI is useful in diagnosing JDM and may be used as a disease assessment tool. Type 1 interferon genes and proteins are increasing in use as disease assessment tools, but larger, prospective, validation studies are needed. Moderate-to-intense physical activity is effective in increasing the aerobic capacity of JDM patients in remission. New criteria developed by the Paediatric Rheumatology International Trials Organization for classifying inactive disease in JDM have practical applicability to the current clinical practice and clinical trials as even after 16.8 years of symptom onset, over half of JDM patients still have active disease. Summary: There has been significant progress in understanding the clinical characteristics, diagnostic workup, treatment, disease assessment, and prognosis of JIIM patients, but more prospective treatment trials are needed, especially in light of the paucity of the current biologic treatment agents available.

AB - Purpose of review: Ongoing research continues to advance our understanding of the juvenile idiopathic inflammatory myopathies (JIIMs). We review the recent contributions from the published literature about the classification, pathogenesis, assessment, and treatment of JIIMs in basic and translational science and clinical research in 2013 through early 2014. Recent findings: Large registries, such as the Childhood Arthritis and Rheumatology Research Alliance registry, are conducting trials to enhance our understanding of JIIMs. Ultraviolet radiation exposure 1 month prior to juvenile dermatomyositis (JDM) may trigger the onset of disease. Myositis-specific autoantibodies define clinical phenotypes in JIIMs. MRI is useful in diagnosing JDM and may be used as a disease assessment tool. Type 1 interferon genes and proteins are increasing in use as disease assessment tools, but larger, prospective, validation studies are needed. Moderate-to-intense physical activity is effective in increasing the aerobic capacity of JDM patients in remission. New criteria developed by the Paediatric Rheumatology International Trials Organization for classifying inactive disease in JDM have practical applicability to the current clinical practice and clinical trials as even after 16.8 years of symptom onset, over half of JDM patients still have active disease. Summary: There has been significant progress in understanding the clinical characteristics, diagnostic workup, treatment, disease assessment, and prognosis of JIIM patients, but more prospective treatment trials are needed, especially in light of the paucity of the current biologic treatment agents available.

KW - Clinical characteristics

KW - Immunopathogenesis

KW - Juvenile dermatomyositis

KW - Prognosis

KW - Therapy

UR - http://www.scopus.com/inward/record.url?scp=84927771647&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84927771647&partnerID=8YFLogxK

U2 - 10.1097/BOR.0000000000000103

DO - 10.1097/BOR.0000000000000103

M3 - Article

C2 - 25160931

AN - SCOPUS:84927771647

VL - 26

SP - 671

EP - 678

JO - Current Opinion in Rheumatology

JF - Current Opinion in Rheumatology

SN - 1040-8711

IS - 6

ER -