Complement defects D84.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Definition
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Congenital defects or dysfunctions of one or more complement components. See tab.

Tables
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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

Incoming links (1)

C1S Gene;

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020