title: Serum SC5b-9 (terminal complement complex) level, a sensitive indicator of disease activity in patients with Henoch-Schönlein purpura.
abstract: The concentration of the terminal complement complex (TCC), SC5b-9, was determined by enzyme immunoassay using 95 serum samples from 30 patients with Henoch-Schönlein purpura (HSP), 27 with other forms of inflammatory skin disease and 20 normal healthy donors. Twenty-five patients with HSP showed significantly increased TCC concentration in the active phase of the disease, during which newly formed urticarial or purpuric macules/papules could be seen. Skin biopsy specimens of skin lesions from patients with elevated TCC levels in nearly all cases contained the membrane attack complex of complement and consisted of C5b, C6, C7, C8, C9 without S protein on the vessel walls. Systemic and local activation of complement may thus possibly occur in HSP. Three patients with various manifestations of the disease were followed over a period of several years during which the active and inactive phases were scanned. TCC elevation in all cases was correlated with exacerbation of the disease. In contrast, C3, C4 and CH50 levels either remained normal or increased and thus were not reliable indicators of disease activity. Measurement of TCC should thus prove quite useful for monitoring the activity of HSP in patients in whom there is complement activation and also serve to facilitate clarification of the functions of complement in the pathogenesis of the disease.
authors.affiliation: Department of Dermatology, St. Luke's International Hospital, Tokyo, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Kawana
authors.forename: S
authors.initials: S
authors.lastname: Nishiyama
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D003167:Complement Activation; D015938:Complement Membrane Attack Complex / Q000032:analysis*; D004730:Endothelium, Vascular / Q000737:chemistry; D015200:Fluoroimmunoassay; D006801:Humans; D011695:IgA Vasculitis / Q000276:immunology* / Q000473:pathology; D007136:Immunoglobulins / Q000032:analysis; D008875:Middle Aged; D012867:Skin / Q000098:blood supply
chemical_list: D015938:Complement Membrane Attack Complex; D007136:Immunoglobulins