Table 1.
Clinical symptoms and laboratory findings
| Initial | 1st year | 2nd year | 5th year | |
|---|---|---|---|---|
| Clinical symptoms | 19/19 | 17/19 | 16/19 | 16/18 |
| Arterial hypertension | 72% (13/18) | 74% (14/19) | 79% (15/19) | 83% (15/18) |
| Hematuria | 75% (6/8) | 40% (6/15) | 38% (5/13) | 21% (3/14) |
| Proteinuria | 75% (6/8) | 78% (14/18) | 73% (11/15) | 44% (7/16) |
| Reduced kidney function | 68% (13/19) | 58% (11/19) | 53% (10/19) | 28% (5/18) |
| Abnormal kidney ultrasound | N/A | 62% (5/8) | 50% (4/8) | 56% (5/9) |
| Abnormal ABPM | N/A | 40% (2/5) | 60% (3/5) | 50% (2/4) |
| Extrarenal manifestation | 58% (11/19) | 21% (4/19) | 5% (1/19) | 11% (2/18) |
| Laboratory examinations | ||||
| Minimum hemoglobin, g/dL | 5.7 ± 1.1 | 11.9 ± 2.4 | 12.3 ± 1.6 | 12.7 ± 1.8 |
| (5.0–6.6) | (10.8–13.7) | (11.4–13.2) | (12.3–13.8) | |
| (N = 18) | (N = 15) | (N = 18) | (N = 18) | |
| Elevated LDH* | 100% (17/17) | 67% (6/9) | 27% (3/11) | 11% (1/9) |
| Decreased haptoglobin* | 88% (7/8) | 40% (2/5) | 60% (3/5) | 0% (0/4) |
| Minimum platelets, 109/L | 29 ± 12 | 265 ± 94 | 269 ± 65 | 278 ± 72 |
| (21–35) | (193–347) | (214–312) | (231–332) | |
| (N = 17) | (N = 14) | (N = 18) | (N = 18) | |
| Maximum creatinine, μmol/L | 390 ± 280 | 222 ± 311 | 217 ± 319 | 145 ± 236 |
| (196–500) | (67–188) | (54–94) | (56–78) | |
| (N = 17) | (N = 18) | (N = 19) | (N = 18) | |
| eGFR, ml/min/1.73 m2 | 75 ± 56 | 82 ± 44 | 87 ± 45 | 105 ± 25 |
| (12–132) | (51–118) | (68–121) | (80–125) | |
| (N = 8) | (N = 13) | (N = 13) | (N = 11)# | |
| CFH-Ab titer, AU/ml | 1533 ± 481 | 604 ± 375 | <100 (N = 2); | <100 (N = 10); |
| (959–1933) | (368–809) | 461 ± 224 | 290 ± 142 | |
| (264–650) | (135–394) | |||
| (N = 10) | (N = 14) | (N = 15) | (N = 8) | |
| C3 level, mg/dL | 76 ± 29 | 93 ± 27 | 87 ± 18 | 100 ± 17 |
| (56–95) | (67–110) | (69–101) | (82–118) | |
| (N = 16) | (N = 12) | (N = 12) | (N = 10) | |
| Low C3 level* | 65% (11/17) | 67% (6/9) | 33% (3/9) | 13% (1/8) |
| C4 level, mg/dL | 21 ± 9.7 | 26 ± 16 | 21 ± 7.5 | 23 ± 12 |
| (12–24) | (13–34) | (17–24) | (13–30) | |
| (N = 12) | (N = 11) | (N = 10) | (N = 9) | |
| Low C4 level* | 14% (2/14) | 14% (1/7) | 0% (0/6) | 0% (0/6) |
The number of evaluable answers is indicated in parentheses (N). Data is presented as absolute number of cases per number of evaluable patients (n/N) or as mean ± standard deviation (interquartile range)
CNS central nervous system, LDH lactate dehydrogenase, eGFR estimated glomerular filtration rate calculated with the Schwartz formula
*According to local references used by the respective laboratory, which was unavailable in several cases
#Excluding the two patients who underwent renal transplantation