Description

An inherited deficiency of a complement component may be associated with one or more recurrent diseases.


 

Disorders:

(1) recurrent pyogenic infections

(2) recurrent infections with Neisseria meningitidis or gonorrhoeae

(3) rheumatic disease (collagen vascular disease)

(4) hereditary angioedema

(5) paroxysmal nocturnal hemoglobinuria (PNH)

 

Hereditary complement deficiencies associated with recurrent pyogenic infections:

(1) C1q, C1r or C1s

(2) C3

(3) C4

(4) C5

(5) Factor H

(6) Factor I

(7) CR3 (complement receptor 3)

 

Hereditary complement deficiencies associated with recurrent Neisseria infections:

(1) C5

(2) C6

(3) C7

(4) C8

(5) C9

(6) Factor D

(7) Properdin

Hereditary Complement Deficiencies

Associated Rheumatic Disease

C1q, C1r or C1s

SLE

C2

SLE, Henoch-Schonlein purpura, dermatomyositis

C3

membranoproliferative glomerulonephritis, allergic vasculitis

C4

SLE

C5

SLE

C6

Raynaud's, cryoglobulinemia

C7

Raynaud's, sclerodactyly

Factor H

membranoproliferative glomerulonephritis

C1-INH (C1 inhibitor)

hereditary angioedema

 

 

Hereditary Deficiency

Other Disease

CD55 (decay accelerating factor), CD59

paroxysmal nocturnal hemoglobinuria

 


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