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Table 10. Clinical Associations with Complement Deficiency
Component(s) Lupus-like disease Bacterial infections
C1, C2, C4 Yes Encapsulated organisms
C3 No Encapsulated organisms
(severe)
C5, C6, C7 Yes Neisseria species
C8, C9 No Neisseria species
C1 inhibitor (SERPING1) No (hereditary
angioedema)
None
Factor B No (atypical HUS) None
Factor D No Encapsulated organisms
Properdin Yes Neisseria species
Factor H No (atypical HUS, macular
degeneration)
Encapsulated organisms
Factor H–related protein
(CFHR1-5)
No (atypical HUS) None
Factor I No (atypical HUS) Encapsulated organisms
MBL No Encapsulated organisms
MASP1 No (3MC syndrome) None
MBL-associated serum
protease 2
No Encapsulated organisms
Ficolin 3 No Encapsulated organisms,
necrotizing enterocolitis
rombomodulin No (atypical HUS) None
Membrane cofactor
protein (CD46)
No (atypical HUS) None
Membrane attack complex
inhibitor (CD59)
No (hemolysis,
polyneuropathy)
None
COLEC11 No (3MC syndrome) None
Complement receptor 2
(CD21)
No, CVID-like disorder Encapsulated organisms
Complement receptor 3
(CD18/ITGB2)
No, LAD type 2 Encapsulated organisms
Complement Deficiencies
Anti-Cytokine Autoantibodies
