Table 3.
Complement pathology.
| Analyses | CP function (%) | AP function (%) |
C1-INH conc (g/L) |
C1-INH funct (%) |
C1q conc (g/L) |
C4 conc (g/L) |
C3 conc (g/L) |
C3d conc (mg/L) |
C3d/C3 | sC5b-9 (μg/L) |
|---|---|---|---|---|---|---|---|---|---|---|
| Reference interval | 80–120 | 50–150 | 0.13–0.29 | 72–129 | 0.07–0.25 | 0.13–0.32 | 0.67–1.29 | <5.3 | <5.3 | <25 |
| Complement deficiencies CP, e.g., C2 deficiency |
9 | 105 | 0.20 | 82 | 0.13 | 0.23 | 0.80 | 3.6 | 4.5 | <25 |
| Complement deficiencies AP, e.g., P deficiency |
97 | 45 | 0.25 | 95 | 0.20 | 0.20 | 0.97 | 4.4 | 4.5 | <25 |
| Complement deficiencies TP, e.g., C6 deficiency |
8 | 12 | 0.17 | 81 | 0.24 | 0.27 | 1.20 | 4.5 | 3.8 | <25 |
| SLE, urticarial vasculitis |
10 | 109 | 0.17 | 79 | 0.06 | 0.08 | 0.65 | 6.3 | 9.7 | 75 |
| Secondary phospholipid syndrome (SLE) | 15 | 103 | 0.20 | 87 | 0.06 | 0.09 | 0.60 | 7.0 | 11.2 | 90 |
| MPGN I | 65 | 45 | 0.18 | 115 | 0.08 | 0.14 | 0.55 | 12 | 22 | 85 |
| AMR | 30 | 65 | 0.22 | 120 | 0.10 | 0.10 | 0.65 | 7 | 11 | 100 |
| C3GN | 15 | 10 | 0.26 | 122 | 0.22 | 0.15 | 0.10 | 18 | 180 | 200 |
| PSGN | 15 | 10 | 0.24 | 120 | 0.24 | 0.31 | 0.10 | 15 | 150 | 175 |
| aHUS | 95 | 105 | 0.19 | 103 | 0.20 | 0.21 | 0.75 | 6.5 | 8.7 | 75 |
| HAE with low C1-INH conc | 100 | 140 | 0.04 | 20 | 0.23 | 0.10 | 1.23 | 4.3 | 3.5 | <25 |
| HAE with dysfunct C1-INH |
111 | 132 | 0.20 | 18 | 0.18 | 0.10 | 1.01 | 3.3 | 3.3 | <25 |
| AAE | 93 | 94 | 0.03 | 11 | 0.05 | 0.09 | 0.80 | 3.9 | 4.9 | <25 |
| Eculizumab | 5 | 10 | 0.23 | 79 | 0.25 | 0.26 | 1.17 | 4.2 | 3.6 | <25 |
CP, classical pathway; AP alternative pathway; C1-INH, C1 inhibitor; TP, termial pathway; SLE, systemic lupus erythematosus; MPGN, membranoproliferative glomerulonephritis; AMR, antibody mediated rejection; C3GN, C3 glomerulonephritis; aHUS, atypical haemolytic uremic syndrome; PSGN, post-streptococcal glomerulonephritis; HAE, hereditary angioedema, AAE, acquired angioedema.