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.

journal_name

Medicine (Baltimore)

journal_title

Medicine

authors

Chaisson NF,Paik J,Orbai AM,Casciola-Rosen L,Fiorentino D,Danoff S,Rosen A

doi

10.1097/MD.0b013e3182606f0b

subject

Has Abstract

pub_date

2012-07-01 00:00:00

pages

220-228

issue

4

eissn

0025-7974

issn

1536-5964

pii

00005792-201207000-00006

journal_volume

91

pub_type

杂志文章,评审

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