Introduction
Methods
Registry description
Clinical diagnoses and parameters
Evaluation of other clinical findings
Statistical analysis
Results
General data of patients
N
| Min | Max | Mean | SD | |
---|---|---|---|---|---|
Age (years old) | 593 | 3 | 89 | 52.7 | 22.4 |
Male | 319 | 3 | 89 | 54.1 | 21.2 |
Female | 274 | 4 | 88 | 51.0 | 23.6 |
Height (cm) | 582 | 94.5 | 186.1 | 157.8 | 12.7 |
Weight (kg) | 581 | 15.2 | 107.6 | 57.0 | 13.8 |
Body mass index (BMI) | 581 | 10.4 | 37.6 | 22.6 | 3.8 |
Proteinuria (g/day) | 429 | 0.0 | 19.4 | 3.7 | 3.2 |
Urinary protein/creatinine ratio | 444 | 0.0 | 32.0 | 4.8 | 4.2 |
Serum creatinine (mg/dl) | 593 | 0.2 | 9.7 | 1.3 | 1.0 |
eGFR (mL/min/1.73 m2) | 588 | 6.4 | 161.3 | 57.8 | 32.1 |
Serum total protein (g/dl) | 592 | 3.3 | 9.9 | 5.8 | 1.0 |
Serum albumin (g/dl) | 592 | 1.1 | 5.0 | 2.9 | 0.8 |
Serum total cholesterol (mg/dl) | 575 | 78.0 | 558.0 | 225.4 | 72.8 |
Systolic blood pressure (mmHg) | 507 | 87.0 | 227.0 | 138.1 | 21.5 |
Diastolic blood pressure (mmHg) | 507 | 38.0 | 114.0 | 77.4 | 13.3 |
Mean blood pressure (mmHg) | 507 | 57.0 | 146.3 | 97.6 | 14.2 |
HbA1c (%) | 366 | 3.8 | 14.2 | 5.7 | 0.8 |
Urinary protein (dipstick test) | Cases | % | ||||
---|---|---|---|---|---|---|
(−) | 16 | 2.7 | ||||
(±) | 20 | 3.4 | ||||
1+ | 48 | 8.1 | ||||
2+ | 132 | 22.3 | ||||
3+ | 258 | 43.5 | ||||
4+ | 119 | 20.1 | ||||
Total | 593 | 100.0 |
Daily proteinuria levels and urinary protein levels according to spot urine tests | |||||
---|---|---|---|---|---|
g/day | Cases | % | g/g Cr | Cases | % |
< 0.3 | 28 | 6.5 | < 0.3 | 18 | 4.1 |
0.3–0.49 | 16 | 3.7 | 0.3–0.49 | 14 | 3.2 |
0.50–0.99 | 44 | 10.3 | 0.50–0.99 | 30 | 6.8 |
1.0–3.49 | 149 | 34.7 | 1.0-3.49 | 159 | 35.8 |
3.5+ | 192 | 44.8 | 3.5+ | 223 | 50.2 |
Total | 429 | 100.0 | Total | 444 | 100.0 |
Hematuria (occult blood grade and red blood cell grade of urinary sediment) | |||||
---|---|---|---|---|---|
OB | Cases | % | /hpf | Cases | % |
(−) | 58 | 9.8 | (−) | 26 | 4.4 |
(±) | 65 | 11.0 | < 5 | 130 | 21.9 |
1+ | 84 | 14.2 | 5–10 | 98 | 16.5 |
2+ | 169 | 28.5 | < 10–30 | 169 | 28.5 |
3+ | 217 | 36.6 | Many | 170 | 28.7 |
Total | 593 | 100 | Total | 593 | 100.0 |
Cases | % | |
---|---|---|
Clinical diagnoses | ||
Nephrotic syndrome | 253 | 42.7 |
Chronic nephritic syndrome | 220 | 37.1 |
Nephrotic syndrome + collagen disease/vasculitis | 44 | 7.4 |
Chronic nephritic syndrome + collagen disease/vasculitis | 34 | 5.7 |
Rapidly progressive nephritic syndrome | 19 | 3.2 |
Acute nephritic syndrome | 12 | 2.0 |
Recurrent hematuria | 5 | 0.8 |
HUS/TTP | 2 | 0.3 |
Acute kidney injury | 2 | 0.3 |
Acute nephritic syndrome + collagen disease/vasculitis | 1 | 0.2 |
Rapidly progressive nephritic syndrome + collagen disease/vasculitis | 1 | 0.2 |
Total | 593 | 100 |
Pathogenesis | ||
Primary (idiopathic) | 332 | 56.0 |
Lupus nephritis | 74 | 12.5 |
Infection-related nephropathy | 72 | 12.1 |
IgA nephropathy | 29 | 4.9 |
Thrombotic microangiopathy | 8 | 1.3 |
Purpura nephritis | 7 | 1.2 |
Diabetic nephropathy | 6 | 1.0 |
Amyloidosis | 4 | 0.7 |
Hypertensive nephrosclerosis | 3 | 0.5 |
MPO-ANCA-positive nephritis | 2 | 0.3 |
Transplanted kidney | 2 | 0.3 |
Others | 54 | 9.1 |
Total | 593 | 100 |
Clinical features and pathogenesis of MPGN
Child | Adult | Elderly |
P
| |
---|---|---|---|---|
N
| 74 | 282 | 237 | |
Age (years old) | 11.9 ± 4.1 | 46.0 ± 13.3 | 73.4 ± 5.7 | < 0.001* |
Male/female | 36/38 | 158/124 | 125/112 | 0.483 |
Body mass index (BMI) | 18.8 ± 3.1 | 23.2 ± 3.6 | 23.1 ± 3.6 | < 0.001* |
Systolic blood pressure (mmHg) | 113.0 ± 15.0 | 135.6 ± 18.7 | 147.8 ± 19.8 | < 0.001* |
Diastolic blood pressure (mmHg) | 64.7 ± 10.8 | 80.0 ± 12.9 | 77.6 ± 12.4 | < 0.001* |
Patients with hypertension at diagnosis | 17.3% | 60.5% | 86.4% | < 0.001* |
Sediment RBC (> 30/hpf, %) | 40.5% | 24.5% | 29.5% | 0.027* |
Proteinuria (g/day) | 1.7 ± 2.1 | 3.8 ± 3.0 | 3.9 ± 3.5 | < 0.001* |
Urinary protein/creatinine ratio | 2.6 ± 3.9 | 4.5 ± 3.6 | 5.7 ± 4.8 | < 0.001* |
Serum creatinine (mg/dl) | 0.71 ± 1.11 | 1.22 ± 0.75 | 1.56 ± 1.08 | < 0.001* |
eGFR (mL/min/1.73 m2) | 106.2 ± 28.9 | 60.4 ± 28.4 | 40.3 ± 18.7 | < 0.001* |
Serum albumin (g/dl) | 3.4 ± 0.9 | 2.9 ± 0.8 | 2.7 ± 0.7 | < 0.001* |
Serum total cholesterol (mg/dl) | 216.9 ± 74.4 | 229.5 ± 77.5 | 223.2 ± 66.2 | 0.354 |
Clinical diagnosis | ||||
Nephrotic syndrome | 11 (14.9%) | 114 (40.4%) | 128 (54.0%) | < 0.001* |
Chronic nephritic syndrome | 49 (66.2%) | 97 (34.4%) | 74 (31.2%) | < 0.001* |
Nephrotic syndrome + collagen disease/vasculitis | 3 (4.1%) | 30 (10.6%) | 11 (4.6%) | 0.017* |
Chronic nephritic syndrome + collagen disease/vasculitis | 6 (8.1%) | 25 (8.9%) | 3 (1.3%) | 0.001* |
RPGN | 1 (1.4%) | 7 (2.5%) | 11 (4.6%) | 0.238 |
Acute nephritic syndrome | 1 (1.4%) | 5 (1.8%) | 6 (2.5%) | 0.753 |
Recurrent hematuria | 2 (2.7%) | 2 (0.7%) | 1 (0.4%) | 0.163 |
HUS/TTP | 1 (1.4%) | 0 (0.0%) | 1 (0.4%) | 0.195 |
Acute kidney injury | 0 (0.0%) | 1 (0.4%) | 1 (0.4%) | 0.859 |
Acute nephritic syndrome + collagen disease/vasculitis | 0 (0.0%) | 0 (0.0%) | 1 (0.4%) | 0.471 |
RPGN + collagen disease/vasculitis | 1 (1.4%) | 7 (2.5%) | 11 (4.6%) | 0.238 |
Pathological diagnosis | ||||
Primary (idiopathic) | 60 (81.1%) | 129 (45.7%) | 143 (60.3%) | < 0.001* |
Lupus nephritis | 8 (10.8%) | 52 (18.4%) | 14 (5.9%) | < 0.001* |
Infection-related nephropathy | 0 (0.0%) | 37 (13.1%) | 35 (14.8%) | 0.002* |
IgA nephropathy | 3 (4.1%) | 20 (7.1%) | 6 (2.5%) | 0.053 |
Thrombotic microangiopathy | 1 (0.0%) | 6 (1.8%) | 3 (1.3%) | 0.495 |
Purpura nephritis | 2 (2.7%) | 3 (1.1%) | 2 (0.8%) | 0.420 |
Diabetic nephropathy | 0 (0.0%) | 1 (0.4%) | 5 (2.1%) | 0.090 |
Amyloidosis | 0 (0.0%) | 2 (0.7%) | 2 (0.8%) | 0.737 |
Hypertensive nephrosclerosis | 0 (0.0%) | 0 (0.0%) | 3 (1.3%) | 0.104 |
MPO-ANCA-positive nephritis | 0 (0.0%) | 1 (0.4%) | 1 (0.4%) | 0.859 |
Transplanted kidney | 0 (0.0%) | 2 (0.7%) | 0 (0.0%) | 0.331 |
Others | 0 (0.0%) | 29 (10.3%) | 23 (9.7%) | 0.017* |
Clinical features of primary MPGN
N
| Min | Max | Mean | SD | |
---|---|---|---|---|---|
Age (years old) | 332 | 3 | 89 | 51.6 | 24.6 |
Male | 185 | 3 | 89 | 53.5 | 23.1 |
Female | 147 | 4 | 87 | 49.2 | 26.4 |
Height (cm) | 328 | 94.5 | 183.0 | 156.3 | 14.3 |
Weight (kg) | 327 | 15.2 | 102.6 | 55.3 | 14.0 |
Body mass index (BMI) | 327 | 10.4 | 34.9 | 22.3 | 3.8 |
Proteinuria (g/day) | 232 | 0.0 | 19.4 | 3.8 | 3.4 |
Urinary protein/creatinine ratio | 250 | 0.0 | 32.0 | 5.0 | 4.6 |
Serum creatinine (mg/dl) | 332 | 0.2 | 9.7 | 1.3 | 1.1 |
eGFR (ml/min/1.73 m2) | 328 | 6.4 | 161.3 | 59.0 | 33.8 |
Serum total protein (g/dl) | 332 | 3.3 | 8.7 | 5.7 | 1.0 |
Serum albumin (g/dl) | 331 | 1.1 | 5.0 | 3.0 | 0.8 |
Serum total cholesterol (mg/dl) | 324 | 78.0 | 558.0 | 230.7 | 73.3 |
Systolic blood pressure (mmHg) | 284 | 87.0 | 197.0 | 137.9 | 22.4 |
Diastolic blood pressure (mmHg) | 284 | 42.0 | 114.0 | 76.1 | 13.4 |
Mean blood pressure (mmHg) | 284 | 57.0 | 135.0 | 96.7 | 14.5 |
HbA1c (%) | 192 | 3.8 | 7.4 | 5.6 | 0.5 |
Child | Adult | Elderly |
P
| |
---|---|---|---|---|
N
| 60 | 129 | 143 | |
Age (years old) | 11.4 ± 4.2 | 45.8 ± 13.7 | 73.6 ± 5.6 | < 0.001* |
Male/Female | 27/33 | 79/50 | 79/64 | 0.111 |
Body mass index (BMI) | 18.9 ± 3.3 | 22.8 ± 3.2 | 23.3 ± 3.7 | < 0.001* |
Systolic blood pressure (mmHg) | 112.8 ± 15.8 | 136.0 ± 18.1 | 148.2 ± 20.3 | < 0.001* |
Diastolic blood pressure (mmHg) | 64.4 ± 11.5 | 79.3 ± 12.5 | 77.3 ± 12.8 | < 0.001* |
Patients with hypertension at diagnosis | 13.3% | 51.2% | 67.8% | < 0.001* |
Sediment RBC (> 30/hpf, %) | 38.3% | 23.3% | 32.2% | 0.078 |
Proteinuria (g/day) | 1.8 ± 2.2 | 4.0 ± 3.2 | 4.2 ± 3.6 | 0.003* |
Urinary protein/creatinine ratio | 2.6 ± 4.2 | 4.5 ± 3.4 | 6.2 ± 5.2 | < 0.001* |
Serum creatinine (mg/dl) | 0.75 ± 1.23 | 1.29 ± 0.85 | 1.56 ± 1.06 | < 0.001* |
eGFR (ml/min/1.73 m2) | 104.2 ± 30.5 | 59.6 ± 28.7 | 40.2 ± 18.6 | < 0.001* |
Serum albumin (g/dl) | 3.5 ± 0.9 | 3.0 ± 0.8 | 2.8 ± 0.7 | < 0.001* |
Serum total cholesterol (mg/dl) | 219.1 ± 73.4 | 237.9 ± 76.1 | 229.3 ± 70.3 | 0.251 |
Clinical diagnosis | ||||
Nephrotic syndrome | 10 (16.7%) | 63 (48.8%) | 90 (62.9%) | < 0.001* |
Chronic nephritic syndrome | 46 (76.7%) | 60 (46.5%) | 42 (29.4%) | < 0.001* |
Rapidly progressive nephritic syndrome | 1 (1.7%) | 3 (2.3%) | 6 (4.2%) | 0.531 |
Acute nephritic syndrome | 0 (0.0%) | 1 (0.8%) | 1 (0.7%) | 0.798 |
Recurrent hematuria | 2 (3.3%) | 1 (0.8%) | 0 (0.0%) | 0.163 |
Acute kidney injury | 0 (0.0%) | 1 (0.8%) | 1 (0.7%) | 0.798 |
Impact of clinical features on renal function
Parameters at diagnosis | Standard b | t |
P
|
---|---|---|---|
(a) MPGN | |||
Systolic blood pressure | − 0.248 | − 4.740 | < 0.001 |
Proteinuria (g/day) | − 0.177 | − 3.255 | 0.001 |
Serum total cholesterol | 0.108 | 1.998 | 0.046 |
R
2
= 0.104; F = 13.021, p < 0.001* | |||
(b) Primary MPGN | |||
Systolic blood pressure | − 0.299 | − 4.254 | < 0.001 |
Proteinuria (g/day) | − 0.216 | − 2.957 | 0.004 |
Serum total cholesterol | 0.147 | 2.030 | 0.044 |
R
2
= 0.157; F = 10.791, p < 0.001* |