Microvascular deposition of complement membrane attack complex in dermatomyositis.
We examined the role of the complement system in the pathogenesis of dermatomyositis. Using an antibody against the neoantigens of the terminal C5b-9 membrane attack complex, we performed immunocytochemical studies that localized this complex to the intramuscular microvasculature (arterioles and capillaries) of muscle biopsy specimens from 10 of 12 patients (83 percent) with childhood dermatomyositis and 5 of 19 patients (26 percent) with adult dermatomyositis. Fifty-two control specimens, including 14 from patients with polymyositis and 12 from patients with denervation atrophy (a condition known to be associated with necrotic capillaries), showed no deposition of membrane attack complex in the microvasculature. These findings indicate that the complement system is deposited, bound, and activated to completion within the intramuscular microvasculature of patients with dermatomyositis. In addition to providing further evidence for the presence of vasculopathy in dermatomyositis, these findings suggest a primary role for complement in mediating vessel injury in the disease, particularly in its childhood form.
D000328:Adult; D001158:Arteries / Q000276:immunology; D002196:Capillaries / Q000276:immunology; D002648:Child; D003182:Complement C5 / Q000276:immunology; D050776:Complement C5b; D015938:Complement Membrane Attack Complex; D003165:Complement System Proteins / Q000032:analysis* / Q000276:immunology; D003882:Dermatomyositis / Q000209:etiology / Q000276:immunology*; D006801:Humans; D009132:Muscles / Q000098:blood supply* / Q000648:ultrastructure
D003182:Complement C5; D015938:Complement Membrane Attack Complex; D050776:Complement C5b; D003165:Complement System Proteins