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: Zahir Ali Shaikh, MD[2], Anmol Pitliya, M.B.B.S. M.D.[3]

Overview

The complement system is a biochemical cascade which helps clear pathogens from an organism. It belongs to the innate immune system. Deficiencies in this cascade can lead to infections and autoimmune diseases. Complement deficiencies can be inherited or acquired (as a result of complement-consuming disease state).

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