Table 2: Complement pathology, differential diagnostics.

AnalysesHAE/AAESLE, urticarial vasculitisPSGNMPGN
II and III
Complement
deficiencies

CPNLLL *
APNN (L)LL *
C1INH (conc)LNNN *
C1INH (funct)LNNN *
C1q(L)LNN *
C4LLNN *
C3NLLL *
C5NL(L) *
Factor BNNLN *
ProperdinNLN *
C3dNHHHN
C3d,g/C3NHHHN

*Variable (dependent on which component is defective); N: normal; L: low; H: high.