Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity

We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distri...

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Autores: Bingham, April, Mamyrova, Gulnara, Rother, Kristina, Oral, Elif, Cochran, Elaine, Premkumar, Ahalya, Kleiner, David, James-Newton, Laura, Targoff, Ira N., Pandey, Janardan P., Carrick, Danielle Mercatante, Sebring, Nancy, O'Hanlon, Terrance P., Ruiz Hidalgo, María José, Turner, Maria, Gordon, Leslie, Laborda Fernández, Jorge, Bauer, Steven R., Blackshear, Perry J., Imundo, Lisa, Miller, Frederick W., Rider, Lisa G.
Tipo de recurso: artículo
Fecha de publicación:2008
País:España
Institución:Universidad de Castilla-La Mancha
Repositorio:RUIdeRA. Repositorio Institucional de la UCLM
OAI Identifier:oai:ruidera.uclm.es:10578/48028
Acceso en línea:https://hdl.handle.net/10578/48028
https://pmc.ncbi.nlm.nih.gov/articles/PMC2674585/
Access Level:acceso abierto
Palabra clave:Adult-onset dermatomyositis (DM)
Autoantibodies
Insulin resistance
Juvenile dermatomyositis (JDM)
Lipodystrophy (LD)
Metabolic abnormalities
Phenotypes
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spelling Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of SeverityBingham, AprilMamyrova, GulnaraRother, KristinaOral, ElifCochran, ElainePremkumar, AhalyaKleiner, DavidJames-Newton, LauraTargoff, Ira N.Pandey, Janardan P.Carrick, Danielle MercatanteSebring, NancyO'Hanlon, Terrance P.Ruiz Hidalgo, María JoséTurner, MariaGordon, LeslieLaborda Fernández, JorgeBauer, Steven R.Blackshear, Perry J.Imundo, LisaMiller, Frederick W.Rider, Lisa G.Adult-onset dermatomyositis (DM)AutoantibodiesInsulin resistanceJuvenile dermatomyositis (JDM)Lipodystrophy (LD)Metabolic abnormalitiesPhenotypesWe describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. LD onset was often delayed, beginning a median of 4.6 years after diagnosis of DM. Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD. Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, a newly described myositis-associated autoantibody, was more associated with generalized LD. Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatohepatitis were frequent in patients with LD, in a gradient of frequency and severity among the 3 sub-phenotypes. Metabolic studies frequently revealed insulin resistance and hypertriglyceridemia in patients with generalized and partial LD. Regional fat loss from the thighs, with relative sparing of fat loss from the medial thighs, was more frequent in generalized than in partial LD and absent from DM patients without LD. Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in our patients. LD is an under-recognized sequela of JDM, and certain DM patients with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development of this complication. High-risk JDM patients should be screened for metabolic abnormalities, which are common in generalized and partial LD and result in much of the LD-associated morbidity. Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM.Lippincott, Williams & Wilkins202620262008info:eu-repo/semantics/articleapplication/pdfapplication/pdfhttps://hdl.handle.net/10578/48028https://pmc.ncbi.nlm.nih.gov/articles/PMC2674585/reponame:RUIdeRA. Repositorio Institucional de la UCLMinstname:Universidad de Castilla-La ManchaInglésinfo:eu-repo/semantics/openAccessoai:ruidera.uclm.es:10578/480282026-05-27T07:36:41Z
dc.title.none.fl_str_mv Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
title Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
spellingShingle Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
Bingham, April
Adult-onset dermatomyositis (DM)
Autoantibodies
Insulin resistance
Juvenile dermatomyositis (JDM)
Lipodystrophy (LD)
Metabolic abnormalities
Phenotypes
title_short Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
title_full Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
title_fullStr Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
title_full_unstemmed Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
title_sort Predictors of Acquired Lipodystrophy in Juvenile-Onset Dermatomyositis and a Gradient of Severity
dc.creator.none.fl_str_mv Bingham, April
Mamyrova, Gulnara
Rother, Kristina
Oral, Elif
Cochran, Elaine
Premkumar, Ahalya
Kleiner, David
James-Newton, Laura
Targoff, Ira N.
Pandey, Janardan P.
Carrick, Danielle Mercatante
Sebring, Nancy
O'Hanlon, Terrance P.
Ruiz Hidalgo, María José
Turner, Maria
Gordon, Leslie
Laborda Fernández, Jorge
Bauer, Steven R.
Blackshear, Perry J.
Imundo, Lisa
Miller, Frederick W.
Rider, Lisa G.
author Bingham, April
author_facet Bingham, April
Mamyrova, Gulnara
Rother, Kristina
Oral, Elif
Cochran, Elaine
Premkumar, Ahalya
Kleiner, David
James-Newton, Laura
Targoff, Ira N.
Pandey, Janardan P.
Carrick, Danielle Mercatante
Sebring, Nancy
O'Hanlon, Terrance P.
Ruiz Hidalgo, María José
Turner, Maria
Gordon, Leslie
Laborda Fernández, Jorge
Bauer, Steven R.
Blackshear, Perry J.
Imundo, Lisa
Miller, Frederick W.
Rider, Lisa G.
author_role author
author2 Mamyrova, Gulnara
Rother, Kristina
Oral, Elif
Cochran, Elaine
Premkumar, Ahalya
Kleiner, David
James-Newton, Laura
Targoff, Ira N.
Pandey, Janardan P.
Carrick, Danielle Mercatante
Sebring, Nancy
O'Hanlon, Terrance P.
Ruiz Hidalgo, María José
Turner, Maria
Gordon, Leslie
Laborda Fernández, Jorge
Bauer, Steven R.
Blackshear, Perry J.
Imundo, Lisa
Miller, Frederick W.
Rider, Lisa G.
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Adult-onset dermatomyositis (DM)
Autoantibodies
Insulin resistance
Juvenile dermatomyositis (JDM)
Lipodystrophy (LD)
Metabolic abnormalities
Phenotypes
topic Adult-onset dermatomyositis (DM)
Autoantibodies
Insulin resistance
Juvenile dermatomyositis (JDM)
Lipodystrophy (LD)
Metabolic abnormalities
Phenotypes
description We describe the clinical features of 28 patients with juvenile dermatomyositis (JDM) and 1 patient with adult-onset dermatomyositis (DM), all of whom developed lipodystrophy (LD) that could be categorized into 1 of 3 phenotypes, generalized, partial, or focal, based on the pattern of fat loss distribution. LD onset was often delayed, beginning a median of 4.6 years after diagnosis of DM. Calcinosis, muscle atrophy, joint contractures, and facial rash were DM disease features found to be associated with LD. Panniculitis was associated with focal lipoatrophy while the anti-p155 autoantibody, a newly described myositis-associated autoantibody, was more associated with generalized LD. Specific LD features such as acanthosis nigricans, hirsutism, fat redistribution, and steatosis/nonalcoholic steatohepatitis were frequent in patients with LD, in a gradient of frequency and severity among the 3 sub-phenotypes. Metabolic studies frequently revealed insulin resistance and hypertriglyceridemia in patients with generalized and partial LD. Regional fat loss from the thighs, with relative sparing of fat loss from the medial thighs, was more frequent in generalized than in partial LD and absent from DM patients without LD. Cytokine polymorphisms, the C3 nephritic factor, insulin receptor antibodies, and lamin mutations did not appear to play a pathogenic role in the development of LD in our patients. LD is an under-recognized sequela of JDM, and certain DM patients with a severe, prolonged clinical course and a high frequency of calcinosis appear to be at greater risk for the development of this complication. High-risk JDM patients should be screened for metabolic abnormalities, which are common in generalized and partial LD and result in much of the LD-associated morbidity. Further study is warranted to investigate the pathogenesis of acquired LD in patients with DM.
publishDate 2008
dc.date.none.fl_str_mv 2008
2026
2026
dc.type.none.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv https://hdl.handle.net/10578/48028
https://pmc.ncbi.nlm.nih.gov/articles/PMC2674585/
url https://hdl.handle.net/10578/48028
https://pmc.ncbi.nlm.nih.gov/articles/PMC2674585/
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv Lippincott, Williams & Wilkins
publisher.none.fl_str_mv Lippincott, Williams & Wilkins
dc.source.none.fl_str_mv reponame:RUIdeRA. Repositorio Institucional de la UCLM
instname:Universidad de Castilla-La Mancha
instname_str Universidad de Castilla-La Mancha
reponame_str RUIdeRA. Repositorio Institucional de la UCLM
collection RUIdeRA. Repositorio Institucional de la UCLM
repository.name.fl_str_mv
repository.mail.fl_str_mv
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