TY - JOUR
T1 - A novel dermato-pulmonary syndrome associated with MDA-5 antibodies
T2 - Report of 2 cases and review of the literature
AU - Chaisson, Neal F.
AU - Paik, Julie
AU - Orbai, Ana Maria
AU - Casciola-Rosen, Livia
AU - Fiorentino, David
AU - Danoff, Sonye
AU - Rosen, Antony
PY - 2012/7
Y1 - 2012/7
N2 - 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.
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.
UR - http://www.scopus.com/inward/record.url?scp=84863719177&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84863719177&partnerID=8YFLogxK
U2 - 10.1097/MD.0b013e3182606f0b
DO - 10.1097/MD.0b013e3182606f0b
M3 - Review article
C2 - 22732950
AN - SCOPUS:84863719177
SN - 0025-7974
VL - 91
SP - 220
EP - 228
JO - Medicine (United States)
JF - Medicine (United States)
IS - 4
ER -