Conditions Associated with Hereditary Complement Deficiencies
Complement Components | Disease |
---|---|
Early (C1, C2, C4) | SLE-like disease |
Glomerulonephritis | |
Mid (C3, C4) | Recurrent pyogenic infections |
SLE-like disease | |
Terminal (C5-C9) | Recurrent infections (especially gonococci and meningococci) |
Regulatory proteins | HAE (C1INH), aHUS (Factor H), AMD (Factor H), PNH (CD55/DAF & CD59) |
aHUS, atypical hemolytic uremic syndrome; AMD, adult macular degeneration; DAF, decay-accelerating factor; HAE, hereditary angioedema; PNH, paroxysmal nocturnal hemoglobinuria; SLE , systemic lupus erythematosus.