Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

Childhood Myositis Heterogeneity Collaborative Study Group

Research output: Contribution to journalArticle

Abstract

Objective. We examined features of clinically amyopathic JDM (CAJDM), in which patients have characteristic rashes with little to no evidence of muscle involvement, to determine whether this is a distinct phenotype from JDM. Methods. Demographic, clinical, laboratory and treatment data from 12 (9 hypomyopathic, 3 amyopathic) patients meeting modified Sontheimer criteria for CAJDM and from 60 matched JDM patients meeting Bohan and Peter criteria were examined. Differences were evaluated by Fisher’s exact and MannWhitney tests, random forests and logistic regression analysis. Results. Nine (75%) CAJDM patients had anti-p155/140 (transcriptional intermediary factor 1), one (8.3%) anti-melanoma differentiation-associated gene 5 autoantibodies and two (16.7%) were myositis autoantibody negative. CAJDM patients were younger at diagnosis and frequently had mild disease at onset. CAJDM patients had less frequent myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain and fatigue. The muscle, skeletal and overall clinical scores were lower in CAJDM. Serum muscle enzymes were less frequently increased in CAJDM, and peak values were lower. CAJDM patients received fewer medications compared with JDM patients. Only 50% of CAJDM patients received oral prednisone, but the maximum dose and treatment duration did not differ from JDM. At a median follow-up of 2.9 years, CAJDM patients had no documented functional disability, and none developed weakness, calcinosis, interstitial lung disease or lipodystrophy. Multivariable modelling revealed a lower skeletal score and less frequent myalgias as the most important factors in distinguishing CAJDM from JDM. Conclusion. CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.

Original languageEnglish (US)
Pages (from-to)1956-1963
Number of pages8
JournalRheumatology (United Kingdom)
Volume57
Issue number11
DOIs
StatePublished - Jan 1 2018

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Autoantibodies
Calcinosis
Myalgia
Amyopathic dermatomyositis
Juvenile dermatomyositis
Lipodystrophy
Muscles
Myositis
Interstitial Lung Diseases
Contracture
Deglutition Disorders
Prednisone
Exanthema
Abdominal Pain
Arthritis
Fatigue
Melanoma
Skeletal Muscle
Therapeutics
Logistic Models

Keywords

  • Classification criteria
  • Clinically amyopathic dermatomyositis
  • Clinically hypomyopathic dermatomyositis
  • Juvenile dermatomyositis
  • Outcome
  • Treatment

ASJC Scopus subject areas

  • Rheumatology
  • Pharmacology (medical)

Cite this

Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis. / Childhood Myositis Heterogeneity Collaborative Study Group.

In: Rheumatology (United Kingdom), Vol. 57, No. 11, 01.01.2018, p. 1956-1963.

Research output: Contribution to journalArticle

Childhood Myositis Heterogeneity Collaborative Study Group. / Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis. In: Rheumatology (United Kingdom). 2018 ; Vol. 57, No. 11. pp. 1956-1963.
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title = "Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis",
abstract = "Objective. We examined features of clinically amyopathic JDM (CAJDM), in which patients have characteristic rashes with little to no evidence of muscle involvement, to determine whether this is a distinct phenotype from JDM. Methods. Demographic, clinical, laboratory and treatment data from 12 (9 hypomyopathic, 3 amyopathic) patients meeting modified Sontheimer criteria for CAJDM and from 60 matched JDM patients meeting Bohan and Peter criteria were examined. Differences were evaluated by Fisher’s exact and MannWhitney tests, random forests and logistic regression analysis. Results. Nine (75{\%}) CAJDM patients had anti-p155/140 (transcriptional intermediary factor 1), one (8.3{\%}) anti-melanoma differentiation-associated gene 5 autoantibodies and two (16.7{\%}) were myositis autoantibody negative. CAJDM patients were younger at diagnosis and frequently had mild disease at onset. CAJDM patients had less frequent myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain and fatigue. The muscle, skeletal and overall clinical scores were lower in CAJDM. Serum muscle enzymes were less frequently increased in CAJDM, and peak values were lower. CAJDM patients received fewer medications compared with JDM patients. Only 50{\%} of CAJDM patients received oral prednisone, but the maximum dose and treatment duration did not differ from JDM. At a median follow-up of 2.9 years, CAJDM patients had no documented functional disability, and none developed weakness, calcinosis, interstitial lung disease or lipodystrophy. Multivariable modelling revealed a lower skeletal score and less frequent myalgias as the most important factors in distinguishing CAJDM from JDM. Conclusion. CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.",
keywords = "Classification criteria, Clinically amyopathic dermatomyositis, Clinically hypomyopathic dermatomyositis, Juvenile dermatomyositis, Outcome, Treatment",
author = "{Childhood Myositis Heterogeneity Collaborative Study Group} and Gulnara Mamyrova and Takayuki Kishi and Targoff, {Ira N.} and Alison Ehrlich and Curiel, {Rodolfo V.} and Rider, {Lisa G.} and Abramson, {Leslie S.} and Bita Arabshahi and Victoria Cartwright and Chalom, {Elizabeth J.} and Eberhardt, {Barbara Anne} and William Hannan and Higgins, {Gloria C.} and Fuhlbrigge, {Robert C.} and Jacobs, {Jerry C.} and Lawrence Jung and Yukiko Kimura and Lindsley, {Carol B.} and Martin, {Alan L.} and Miller, {Frederick W.} and Diana Milojevic and Ostrov, {Barbara E.} and Perez, {Maria D.} and Rivas-Chacon, {Rafael F.} and Margalit Rosenkranz and Sherry, {David D.} and Jennifer Soep and Sangeeta Sule and Vogelgesang, {Scott A.}",
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volume = "57",
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T1 - Features distinguishing clinically amyopathic juvenile dermatomyositis from juvenile dermatomyositis

AU - Childhood Myositis Heterogeneity Collaborative Study Group

AU - Mamyrova, Gulnara

AU - Kishi, Takayuki

AU - Targoff, Ira N.

AU - Ehrlich, Alison

AU - Curiel, Rodolfo V.

AU - Rider, Lisa G.

AU - Abramson, Leslie S.

AU - Arabshahi, Bita

AU - Cartwright, Victoria

AU - Chalom, Elizabeth J.

AU - Eberhardt, Barbara Anne

AU - Hannan, William

AU - Higgins, Gloria C.

AU - Fuhlbrigge, Robert C.

AU - Jacobs, Jerry C.

AU - Jung, Lawrence

AU - Kimura, Yukiko

AU - Lindsley, Carol B.

AU - Martin, Alan L.

AU - Miller, Frederick W.

AU - Milojevic, Diana

AU - Ostrov, Barbara E.

AU - Perez, Maria D.

AU - Rivas-Chacon, Rafael F.

AU - Rosenkranz, Margalit

AU - Sherry, David D.

AU - Soep, Jennifer

AU - Sule, Sangeeta

AU - Vogelgesang, Scott A.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - Objective. We examined features of clinically amyopathic JDM (CAJDM), in which patients have characteristic rashes with little to no evidence of muscle involvement, to determine whether this is a distinct phenotype from JDM. Methods. Demographic, clinical, laboratory and treatment data from 12 (9 hypomyopathic, 3 amyopathic) patients meeting modified Sontheimer criteria for CAJDM and from 60 matched JDM patients meeting Bohan and Peter criteria were examined. Differences were evaluated by Fisher’s exact and MannWhitney tests, random forests and logistic regression analysis. Results. Nine (75%) CAJDM patients had anti-p155/140 (transcriptional intermediary factor 1), one (8.3%) anti-melanoma differentiation-associated gene 5 autoantibodies and two (16.7%) were myositis autoantibody negative. CAJDM patients were younger at diagnosis and frequently had mild disease at onset. CAJDM patients had less frequent myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain and fatigue. The muscle, skeletal and overall clinical scores were lower in CAJDM. Serum muscle enzymes were less frequently increased in CAJDM, and peak values were lower. CAJDM patients received fewer medications compared with JDM patients. Only 50% of CAJDM patients received oral prednisone, but the maximum dose and treatment duration did not differ from JDM. At a median follow-up of 2.9 years, CAJDM patients had no documented functional disability, and none developed weakness, calcinosis, interstitial lung disease or lipodystrophy. Multivariable modelling revealed a lower skeletal score and less frequent myalgias as the most important factors in distinguishing CAJDM from JDM. Conclusion. CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.

AB - Objective. We examined features of clinically amyopathic JDM (CAJDM), in which patients have characteristic rashes with little to no evidence of muscle involvement, to determine whether this is a distinct phenotype from JDM. Methods. Demographic, clinical, laboratory and treatment data from 12 (9 hypomyopathic, 3 amyopathic) patients meeting modified Sontheimer criteria for CAJDM and from 60 matched JDM patients meeting Bohan and Peter criteria were examined. Differences were evaluated by Fisher’s exact and MannWhitney tests, random forests and logistic regression analysis. Results. Nine (75%) CAJDM patients had anti-p155/140 (transcriptional intermediary factor 1), one (8.3%) anti-melanoma differentiation-associated gene 5 autoantibodies and two (16.7%) were myositis autoantibody negative. CAJDM patients were younger at diagnosis and frequently had mild disease at onset. CAJDM patients had less frequent myalgias, arthritis, contractures, calcinosis, dysphagia, abdominal pain and fatigue. The muscle, skeletal and overall clinical scores were lower in CAJDM. Serum muscle enzymes were less frequently increased in CAJDM, and peak values were lower. CAJDM patients received fewer medications compared with JDM patients. Only 50% of CAJDM patients received oral prednisone, but the maximum dose and treatment duration did not differ from JDM. At a median follow-up of 2.9 years, CAJDM patients had no documented functional disability, and none developed weakness, calcinosis, interstitial lung disease or lipodystrophy. Multivariable modelling revealed a lower skeletal score and less frequent myalgias as the most important factors in distinguishing CAJDM from JDM. Conclusion. CAJDM may be distinguished from JDM, in that they often have p155/140 (transcriptional intermediary factor 1) autoantibodies, have fewer systemic manifestations and receive less therapy.

KW - Classification criteria

KW - Clinically amyopathic dermatomyositis

KW - Clinically hypomyopathic dermatomyositis

KW - Juvenile dermatomyositis

KW - Outcome

KW - Treatment

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DO - 10.1093/rheumatology/key190

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VL - 57

SP - 1956

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JF - Rheumatology

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