Table 2.
Baseline demographic and clinical characteristics of 40 children with ANCA GN from 1987 to 2012
| Characteristic | Focal ( n=13) | Crescentic/Mixed (n=22) | Sclerotic (n=5) | P Value |
|---|---|---|---|---|
| Demographic | ||||
| Male | 2 | 9 | 1 | 0.74 |
| Age at ANCA GN diagnosis (yr) | 13.5 (7–15) | 11 (8–15) | 11.2 (11.1–13.2) | 0.84 |
| Time to ANCA GN Diagnosis (d) | 109 (34–163) | 26 (10–51) | 24 (14–86) | 0.16 |
| Diagnosis | ||||
| MPA | 4 (30.7) | 13 (59.1) | 4 (80) | 0.05 |
| GPA | 8 (66.7) | 10 (43.5) | 1 (20) | 0.05 |
| ANCA positive | 9 (81.8) | 18 (85.7) | 4 (80) | 0.79 |
| ANCA specificity | ||||
| p-ANCA | 4 (36.4) | 11 (57.9) | 4 (80) | 0.29 |
| c-ANCA | 5 (45.5) | 6 (31.6) | 0 | — |
| PR3 | 5 (55.5) | 10 (62.5) | 3 (100) | 0.37 |
| MPO | 4 (44.5) | 6 (37.5) | 0 (0) | |
| ELISA titer | ||||
| 1/80 | 2 (22.2) | 3 (17.7) | 0 | 0.76 |
| 1/160 | 2 (22.2) | 6 (35.3) | 1 (25) | – |
| 1/320 | 3 (33.3) | 2 (11.8) | 1 (25) | – |
| 1/640 | 2 (22.2) | 6 (35.3) | 2 (50) | – |
| Kidney status at presentation | ||||
| Creatinine (mg/dl) | 0.6 (0.5–0.9) | 2.8 (1.1–6.4) | 3.5 (3.1–9.7) | 0.01 |
| eGFR (ml/min per 1.73 m2) | 106.3 (84.8–126.6) | 19.6 (10.7–57.7) | 19.4 (15–20.0) | <0.001 |
| Proteinuria non-nephrotic (n) | 10 | 12 | 1 | 0.01 |
| Nephrotic (n) | 1 | 8 | 2 | – |
| Protein-to-creatinine ratio (mg/g) | 695 (265–1425) | 1894 (1327–3735) | 24,925 (13,000–36,849) | 0.02 |
| Need for dialysis at diagnosis | 0 | 9 (39) | 3 (60) | 0.01 |
| Other | ||||
| Hemoglobin (g/dL) | 11.7 (11.0–11.8) | 9.85 (8.1–10.8) | 8.5 (6.5–9.3) | 0.02 |
| Platelets (×109/L) | 291 (240–355) | 361 (294–416) | 391 (238–438) | 0.42 |
| Neutrophils (×109/L) | 7.4 (4.4–8.8) | 9.7 (6.8–12.6) | 5.5 (4.4–9.6) | 0.23 |
| Albumin (g/dl) | 3.6 (3.5–3.9) | 3.1 (2.7–3.4) | 2.6 (2.6–3.1) | 0.01 |
| ESR (mm/hr) | 32 (12–90) | 103 (54–133) | 93 (78–110) | 0.02 |
| CRP (mg/L) | 3.4 (0.9–22.2) | 55.5 (7.3–183) | 59.3 (0.6–118) | 0.32 |
| C3 (g/L) | 1.2 (0.99–1.3) | 1.3 (1.1–1.5) | 0.97 (0.85–1.0) | 0.39 |
| IgA (mg/dl) | 150 (120–200) | 150 (100–280) | 220 (130–290) | 0.71 |
| IgM (mg/dl) | 120 (80–170) | 90 (60–130) | 125 (120–150) | 0.53 |
| IgG (mg/dl) | 1050 (720–1330) | 990 (620–1490) | 1300 (920–1590) | 0.79 |
| Treatment of ANCA GN | ||||
| Corticosteroids | 11 (84.6) | 22 (100) | 5 (100) | 0.14 |
| Cyclophosphamide | 5 (38.5) | 17 (77.3) | 2 (40) | 0.05 |
| Plasma exchange | 1 (7.7) | 4 (18.1) | 1 (10) | 0.68 |
| Azathioprine | 2 (15.4) | 0 | 0 | 0.12 |
| Methotrexate | 4 (30.8) | 0 | 0 | 0.03 |
| Rituximab | 2 (15.4) | 0 | 0 | 0.12 |
Continuous variables were analyzed using one-way ANOVA and categorical variables by chi- square analyses. Unless otherwise noted, values are expressed as number (percentage) of patients or median (range). MPA. microscopic polyangiitis, GPA, granulomatosis with polyangiitis; p-ANCA, perinuclear ANCA; c-ANCA, cytoplasmic ANCA; PR3, proteinase 3; MPO, myeloperoxidase; ESR, erythrocyte sedimentation rate; CRP, C-reactive protein.