COMPLEMENT - DEFICIENCY AND ASSOCIATED DISEASE Flashcards
SLE- like syndrome; decrease secondary to a gamma globulinemia
C1q
SLE- like syndrome; dermatomyositis, vasculitis, recurrent infections and chronic glomerulo-nephritis, necrotizing skin lesions, arthritis
C1r
SLE, SLE-like syndrome
C1s
Hereditary angioedema, lupus
nephritis
C1 INH
Recurrent pyogenic infections, SLE, SLE-like syndrome,
discoid lupus, membranoproliferative glomerulonephritis, dermatomyositis, synovitis, purpura, Henoch- Schonlein purpura, hypertension, Hodgkin’s disease, chronic lymphocytic leukemia, dermatitis herpetiformis, polymyositis
C2
Recurrent pyogenic infections, SLE-like syndrome, arthralgias, skin rash
C3
Recurrent pyogenic infections, urticaria
C3 inactivator
SLE-like syndrome, SLE, dermatomyositis- like syndrome, vasculitis
C4
Neisseria infections, SLE
C5
Leiner’s disease, gram-negative skin and bowel infection
C5 dysfunction
Neisseria infections, SLE, Raynaud’s phenomenon,
scleroderma-like syndrome, vasculitis
C6
Neisseria infections, SLE, Raynaud’s phenomenon, scleroderma-like syndrome, vasculitis
C7
Neisseria infections, xeroderma pigmentosa, SLE like syndrome
C8