A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: Report of 2 cases and review of the literature

Research output: Contribution to journalArticle

Abstract

Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.

Original languageEnglish (US)
Pages (from-to)220-228
Number of pages9
JournalMedicine (United States)
Volume91
Issue number4
DOIs
StatePublished - Jul 2012

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Lung
Antibodies
Dermatomyositis
Interstitial Lung Diseases
Muscular Diseases
Exanthema
Autoantibodies
Biomarkers
Phenotype
Skin
Population
IFIH1 Interferon-Induced Helicase
Therapeutics

ASJC Scopus subject areas

  • Medicine(all)

Cite this

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title = "A novel dermato-pulmonary syndrome associated with MDA-5 antibodies: Report of 2 cases and review of the literature",
abstract = "Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.",
author = "Chaisson, {Neal F.} and Julie Paik and Ana-Maria Orbai and {Casciola Rosen}, {Livia A} and David Fiorentino and Sonye Danoff and Antony Rosen",
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AU - Chaisson, Neal F.

AU - Paik, Julie

AU - Orbai, Ana-Maria

AU - Casciola Rosen, Livia A

AU - Fiorentino, David

AU - Danoff, Sonye

AU - Rosen, Antony

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AB - Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.

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