Differential immunohistological features of inflammatory myopathies and dysferlinopathy.
This study was performed in order to characterize the types of the infiltrating cells, and the expression profiles of major histocompatibility complex (MHC) class I and membrane attack complex (MAC) in patients with inflammatory myopathies and dysferlinopathy. Immunohistochemical stains were performed using monoclonal antibodies against several inflammatory cell types, MHC class I, and MAC in muscles from inflammatory myopathies and dysferlinopathy. There was significant difference in the types of infiltrating cells between polymyositis (PM), dermatomyositis (DM), and dysferlinopathy, including significantly high CD4+/CD8+ T cell ratio and B/T cell ratio in DM. In dysferlinopathy, CD4+ T cells were the most abundant and the proportions of infiltrating cell types were similar to those of DM. MHC class I was expressed in muscle fibers of PM and DM regardless of the presence of inflammatory infiltrates. MAC was expressed in necrotic fibers and vessels of PM and DM. One patient with early stage DM had a MAC deposits on endomysial capillaries. In dysferlinopathy, MAC deposit was also observed on the sarcolemma of nonnecrotic fibers. The analysis of inflammatory cells, MHC class I expressions and MAC deposits may help to differentiate dysferlinopathy from idiopathic inflammatory myopathy.
Choi
Jae-Hwan
JH
Department of Neurology, Pusan National University School of Medicine, Yangsan, Korea.
Park
Yeong-Eun
YE
Kim
Sung-Il
SI
Kim
Jeong-Il
JI
Lee
Chang-Hoon
CH
Park
Kyu-Hyun
KH
Kim
Dae-Seong
DS
D000328:Adult; D000368:Aged; D003882:Dermatomyositis / Q000276:immunology / Q000473:pathology; D000073939:Dysferlin; D005260:Female; D005805:Genes, MHC Class I; D006801:Humans; D008297:Male; D008565:Membrane Proteins / Q000235:genetics / Q000276:immunology; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000166:cytology / Q000276:immunology / Q000473:pathology; D009124:Muscle Proteins / Q000235:genetics / Q000276:immunology; D049288:Muscular Dystrophies, Limb-Girdle / Q000276:immunology / Q000473:pathology; D009220:Myositis / Q000276:immunology / Q000473:pathology; D017285:Polymyositis / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000166:cytology / Q000276:immunology / Q000473:pathology; D055815:Young Adult
C114839:DYSF protein, human; D000073939:Dysferlin; D008565:Membrane Proteins; D009124:Muscle Proteins