The relationship of complement-mediated microvasculopathy to the histologic features and clinical duration of disease in dermatomyositis
J. T. Kissel, R. K. Halterman, K. W. Rammohan and J. R. Mendell
Department of Neurology, Ohio State University, Columbus 43210.
Accumulating evidence indicates that a complement-mediated
microvasculopathy may play a pathogenic role in dermatomyositis. In a
previous study, we demonstrated neoantigens of the C5b-9 complement
membrane attack complex in the muscle microvasculature of childhood and
adult cases of dermatomyositis. To further characterize the relationship
between the vascular complement deposits and histologic changes,
quantitative histopathologic analyses were performed on 39 dermatomyositis
biopsy specimens (26 adult, 13 children). There was a significant
correlation between the percentage of fascicles with fibers having focal
myofibrillar loss, a change seen early in the evolution of ischemic muscle
fiber damage, and the percentage of fascicles having capillary deposits of
membrane attack complex. Conversely, in biopsy specimens with a higher
percentage of fascicles with perifascicular atrophy, membrane attack
complex deposits were significantly less common. A fascicle-by-fascicle
analysis supported these observations. Patients whose biopsy specimens were
negative for microvascular membrane attack complex had clinical weakness
for a significantly longer time than those patients with vascular
complement deposits. These data support the hypothesis that the
complement-mediated vasculopathy is a primary immunopathogenic event in the
evolution of muscle lesions in dermatomyositis.
Proposed immunologic models of the inflammatory myopathies and potential therapeutic implications
Greenberg
Neurology 2007;69:2008-2019.
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Expression profile of FSHD supports a link between retinal vasculopathy and muscular dystrophy
Osborne et al.
Neurology 2007;68:569-577.
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Diagnostic Significance of Membrane Attack Complex and Vitronectin in Childhood Dermatomyositis
Sakuta et al.
J Child Neurol 2005;20:597-602.
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Greenberg et al.
Neurology 2002;59:1170-1182.
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Miller et al.
J. Neurol. Neurosurg. Psychiatry 2002;73:420-428.
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Gene Expression Profiling in DQA1*0501+ Children with Untreated Dermatomyositis: A Novel Model of Pathogenesis
Tezak et al.
J. Immunol. 2002;168:4154-4163.
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Complement Activation Promotes Muscle Inflammation during Modified Muscle Use
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Am. J. Pathol. 2000;156:2103-2110.
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Expression of HLA Class I Antigens in Skeletal Muscle Is a Diagnostic Marker in Juvenile Dermatomyositis
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J Child Neurol 1997;12:60-63.
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Cardiac and Central Nervous System Vasculitis in a Child With Dermatomyositis
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Proton Magnetic Resonance Imaging and Spectroscopic Studies of the Pathogenesis and Treatment of Juvenile Dermatomyositis
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