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A Novel Dermato-Pulmonary Syndrome Associated With MDA-5 Antibodies Report of 2 Cases and Review of the Literature MEDICINE Chaisson, N. F., Paik, J., Orbai, A., Casciola-Rosen, L., Fiorentino, D., Danoff, S., Rosen, A. 2012; 91 (4): 220-228

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.

View details for DOI 10.1097/MD.0b013e3182606f0b

View details for Web of Science ID 000306113300006

View details for PubMedID 22732950