期刊
MEDICINE
卷 91, 期 4, 页码 220-228出版社
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MD.0b013e3182606f0b
关键词
-
资金
- National Institutes of Health [AR44684, P30 AR053503, T32 AR048522, T32 HL07534]
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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