TY - JOUR
T1 - Myositis-specific autoantibodies: their clinical and pathogenic significance in disease expression
AU - Gunawardena, Harsha
AU - Betteridge, Zoe E
AU - McHugh, Neil J
PY - 2009
Y1 - 2009
N2 - The idiopathic inflammatory myopathies (IIMs)DM and PMhave been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease, internal organ involvement and an association with cancer in adults. Using a clinico-serological approach, DM and PM can be defined into more homogeneous subsets. Over the last few years, myositis-specific autoantibodies (MSAs) have been better characterized including autoantibodies directed against the aminoacyl tRNA-synthetase enzymes, the signal-recognition particle and the Mi-2 protein. In addition, clinically significant novel autoantibodiesanti-CADM-140, anti-SAE (small ubiquitin-like modifier activating enzyme), anti-p155/140 and anti-p140have been described in the adult and juvenile disease spectrum. MSAs are directed against cytoplasmic or nuclear components involved in key regulatory intracellular processes including protein synthesis, translocation and gene transcription. The striking association between unique serological profiles and distinct clinical phenotypes suggests that target autoantigens may play a role in disease induction and propagation. In this review, we discuss the clinical utility and pathogenic significance of MSAs in disease expression.
AB - The idiopathic inflammatory myopathies (IIMs)DM and PMhave been historically defined by broad clinical and pathological criteria. These conditions affect both adults and children with clinical features including muscle weakness, skin disease, internal organ involvement and an association with cancer in adults. Using a clinico-serological approach, DM and PM can be defined into more homogeneous subsets. Over the last few years, myositis-specific autoantibodies (MSAs) have been better characterized including autoantibodies directed against the aminoacyl tRNA-synthetase enzymes, the signal-recognition particle and the Mi-2 protein. In addition, clinically significant novel autoantibodiesanti-CADM-140, anti-SAE (small ubiquitin-like modifier activating enzyme), anti-p155/140 and anti-p140have been described in the adult and juvenile disease spectrum. MSAs are directed against cytoplasmic or nuclear components involved in key regulatory intracellular processes including protein synthesis, translocation and gene transcription. The striking association between unique serological profiles and distinct clinical phenotypes suggests that target autoantigens may play a role in disease induction and propagation. In this review, we discuss the clinical utility and pathogenic significance of MSAs in disease expression.
KW - Dermatomyositis
KW - Myositis-specific autoantibodies
KW - Polymyositis
KW - Inflammatory myopathy
UR - http://www.scopus.com/inward/record.url?scp=65849340426&partnerID=8YFLogxK
UR - http://dx.doi.org/10.1093/rheumatology/kep078
U2 - 10.1093/rheumatology/kep078
DO - 10.1093/rheumatology/kep078
M3 - Article
SN - 1462-0324
VL - 48
SP - 607
EP - 612
JO - Rheumatology
JF - Rheumatology
IS - 6
ER -