Complement profiles in acute post-streptococcal glomerulonephritis.
It is well known that the hypocomplementemia of acute post-streptococcal glomerulonephritis (APSGN) is characterized by markedly reduced serum concentrations of C3 and moderately reduced levels of C5 and properdin (P). However, the extent of the activation of the classical pathway is not well defined and only limited data are available concerning serum concentrations of terminal components other than C5. In serial serum specimens from 14 children with APSGN, the presence and extent of C4 activation was directly assessed by measurement by rocket immunoelectrophoresis for C4 and C4 (C4d/C4 ratio). Elevated values for this ratio, indicating C4 activation, were found in 8 of 14 of the initial serum specimens, and in some patients the ratio remained elevated for several weeks. In contrast, the serum C4 level was low in only 1 specimen (the specimen with the highest C4d/C4 ratio). However, in 10 patients C4 concentrations within the normal range rose in serial serum specimens. Serum C2 concentrations were depressed in the initial specimens from 5 patients. The concentrations of 13 other complement component and control proteins were also measured in these specimens. Levels of terminal components, other than C5, in the initial serum specimens were normal except for depressed C8 in 3 of 13 patients and depressed C6 in 1 of 14. Of these 4 individuals, 3 had the lowest C3 levels in the study. It is concluded that the classical complement pathway is frequently activated in patients with APSGN early in the condition and that subtle abnormalities in C6 and C8 levels occasionally occur.
Wyatt
R J
RJ
Department of Pediatrics, University of Tennessee, Memphis.
Forristal
J
J
West
C D
CD
Sugimoto
S
S
Curd
J G
JG
D000293:Adolescent; D002648:Child; D003181:Complement C4 / Q000378:metabolism; D003171:Complement Pathway, Classical; D003165:Complement System Proteins / Q000378:metabolism*; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology / Q000276:immunology*; D006801:Humans; D008297:Male; D013290:Streptococcal Infections / Q000150:complications*
D003181:Complement C4; D003165:Complement System Proteins