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 |
Purpose: To evaluate a patient for a hereditary complement deficiency based on recurrent clinical findings.
Specialty: Immunology/Rheumatology
Objective: complications
ICD-10: D84.1,