Complement defectsD84.1
DefinitionThis section has been translated automatically.
TablesThis section has been translated automatically.
Most common congenital defects of the complement system
Defective complement component |
Probable mode of inheritance |
Associated clinical symptoms |
C1Q |
unknown |
Recurrent infections, vasculitis, CGN |
C1Q dysfunction |
AR (CD) |
SLE Syndrome |
C1R |
AR |
SLE syndrome, CGN |
C1S |
unknown |
SLE, CGN |
C1S |
unknown |
SLE |
C4 |
AR (CD) |
SLE, vasculitis CDLE |
C2 |
AR (CD) |
SLE, MPGM, purpura rheumatica, dermatomyositis, pneumococcal sepsis |
C3 |
AR (CD) |
CGN, pyogenic infections with missing granulocytosis |
C5 |
AR (CD) |
SLE, pyogenic infections, gonococcal, meningococcal infections |
C5 dysfunction |
AD |
Pyoderma, Sepsis, Erythrodermia desquamativa Leiner |
C6 |
AR (CD) |
Gonococcal and meningococcal infections, SLE syndrome |
C7 |
AR (CD) |
Sclerodactyly, CGN, gonococcal and meningococcal infections |
C8 |
AR (CD) |
Gonococcal and meningococcal infections, SLE syndrome |
C9 |
AR (CD) |
Meningococcal infections |
C1 INH |
AD |
hereditary angioedema |
C3B INA |
AR (CD) |
Pyogenic infections |