Complement deficiencies

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Overview
Classification
Immunodeficiency Affecting Cellular and Humoral Immunity
Combined Immunodeficiency
Predominantly Antibody Deficiency
Diseases of Immune Dysregulation
Congenital Defects of Phagocytes
Defects in Intrinsic and Innate Immunity
Auto-inflammatory Disorders
Complement Deficiencies
Phenocopies of Primary Immunodeficiency

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Template:Zahir, Anmol Pitliya, M.B.B.S. M.D.[2]

Classification

Complement Deficiencies

Susceptibility to Infections

HIGH

LOW

Disseminated Nisserial Infections

Recurrent Pyogenic Infections

SLE like syndrome

Atypical hemolytic uremic syndrome

Others

Absent CH50 & AH50 hemolytic activity, defective bacterial activity

Normal CH50, Absent AH50 hemolytic activity

C3LOF,C3,AR

C1q def: C1QA, C1QB, C1QC

C3GOF, C3, AD

C1 inhibitor SERPING1, AD

C5 def:,C5

Properdin def:, PFC, XL

MASP2 def:, MASP2, AR

C1r def:

FactorB, GOF, CFB, AD

Membrane attack complex inhibitor def:, CD59

C6 def:, C6

Factor D def:, CFD, AR

Fincolin3 def:, FCN3, AR

C1s def:

Factor H def:, CFH, AD or AR

CD55 def:, (CHAPLE disease), AR

C7 def:, C7+vasculitis

Factor B, CFB, LOF, AR

C2 def:

Factor H related protein def:, CFHR1-5, AR, AD

C8 def:, C8A, C8B, C8G

C4 def:, C4A, C4B, AR

Factor I def:, AR

C9 def:, C9 mild susceptibility

Thrombomodulin def:, THBD, AD

Membrane cofactor protein def:, CD46, AD