Table 1 Complement deficiencies
From: Monogenic forms of systemic lupus erythematosus: new insights into SLE pathogenesis
Complement deficiency | Locus | Inheritance pattern | Clinical manifestations | Infection susceptibility |
|---|---|---|---|---|
C1q | 1p36.3-p34.1 | AR | Nephritis, CNS involvement, photosensitivity | Encapsulated bacteria |
C1r/C1s | 12p13 | AR | Nephritis | Encapsulated bacteria |
C4 | 6p21.3 | AR | Multiorgan involvement; glomerulonephritis | Encapsulated bacteria |
C2 | 6p21.3 | AR | Photosensitivity and articular involvement; mild or absent renal, neurological or pleuropericardial involvement | Pyogenic infections; encapsulated bacteria; Streptococcus pneumoniae sepsis and meningitis |
C3 | 19q13 | AR | Malar rash, photosensitivity, arthralgia and Raynaud’s phenomenon | Recurrent pyogenic infections |
C5-C9: MAC | C5/9p34.1, C6-C7/5p13, C8A-C8B/1p32, C8G/9, C9/5p13 | AR | Multiorgan involvement | Neisserial infections |