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Lippincott, Williams & Wilkins, Medicine, 4(91), p. 220-228, 2012

DOI: 10.1097/md.0b013e3182606f0b

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A Novel Dermato-Pulmonary Syndrome Associated With MDA-5 Antibodies

Journal article published in 2012 by Neal F. Chaisson, Julie Paik, Ana-Maria Orbai ORCID, Livia Casciola-Rosen, David Fiorentino, Sonye Danoff ORCID, Antony Rosen
This paper is made freely available by the publisher.
This paper is made freely available by the publisher.

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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.