Conditions Associated with Hereditary Complement Deficiencies

Conditions Associated with Hereditary Complement Deficiencies

Complement ComponentsDisease
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 proteinsHAE (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.

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