Table 1.
Pediatric chronic primary systemic vasculitis (PSV) cohort.
| Small Vessel (n = 53) |
Medium Vessel (n = 16) |
Large Vessel (n = 21) |
|
|---|---|---|---|
| PSV subtype, n (%) | |||
| GPA/limGPA | 43 (81.1) | - | - |
| MPA | 5 (9.4) | - | - |
| EGPA | 2 (3.8) | - | - |
| ANCA + GN | 1 (1.9) | - | - |
| PAN/cPAN | - | 14 (87.5) | - |
| TA | - | - | 19 (90.5) |
| UPV | 2 (3.8) | 2 (12.5) | 2 (9.5) |
| Sex, n (%) | |||
| Female | 35 (66.0) | 10 (62.5) | 15 (71.4) |
| Male | 18 (34.0) | 6 (37.5) | 6 (28.6) |
| No significant difference in representation of males/females between groups (p = 0.8400) based on chi-squared test | |||
| Age (years) of symptom onset—median (range) | * 14.2 (1.9–17.3) | 11.2 (2.7–15.8) | 12.6 (3.7–17.1) |
| 1 ANCA Antigen Positivity, n (%) | |||
| Proteinase 3 (PR3) | 29 (55.8) | 1 (6.7) | - |
| Myeloperoxidase (MPO) | 18 (34.6) | - | - |
| PR3 and MPO | 2 (3.8) | - | - |
| Neither | 3 (5.8) | 14 (93.3) | 18 (100) |
| Disease activity (pVAS) at diagnosis | |||
| Total pVAS, median (range) | 19 (6–50) | 8 (1–18) | 16 (6–26) |
| Subcomponent pVAS, median (range) | |||
| Renal | 10 (0–12) | 0 (0–6) | 4 (0–12) |
| Cardiovascular | 0 (0–4) | 0 | 4 (0–6) |
| Pulmonary | 0 (0–6) | 0 (0–4) | 0 (0–6) |
| Upper respiratory tract | 4 (0–6) | 0 (0–6) | 0 (0–4) |
| 2 Induction Treatment, n (%) | |||
| 3 Immune-suppressing agents | 38 (73.0) | 4 (25.0) | 2 (9.5) |
| 4 Disease-modifying agents | 6 (11.5) | 6 (37.5) | 11 (52.4) |
| 5 Biologic agents | - | 1 (6.3) | 6 (28.6) |
| Corticosteroids | 48 (92.3) | 12 (75.0) | 20 (95.2) |
| 2 Maintenance Treatment, n (%) | |||
| 3 Immune-suppressing agents | 16 (37.2) | 4 (28.6) | 4 (20.0) |
| 4 Disease-modifying agents | 14 (32.5) | 9 (64.3) | 10 (50.0) |
| 5 Biologic agents | - | 2 (14.3) | 8 (40.0) |
| Corticosteroids | 41 (95.3) | 10 (71.4) | 19 (95.0) |
1 Percentage of patients with ANCA calculated based on available data for 52/53 SVV cases, 15/16 MVV cases and 18/21 LVV cases, 2 Percentage of patients on each induction treatment (for 3–6 months following diagnosis) is calculated based on available data within the SVV (52/53), MVV (16/16), and LVV (21/21) groups and likewise for maintenance treatment (initiated after induction treatment is complete) within the SVV (43/53), MVV (14/16), and LVV (20/21) groups. 3 Immune-suppressing agents are most commonly cyclophosphamide and rituximab. 4 Disease-modifying agents are most commonly azathioprine, methotrexate, and mycophenolate mofetil. 5 Biologic agents are most commonly infliximab and tocilizumab. GPA, granulomatosis with polyangiitis; limGPA, limited GPA; MPA, microscopic polyangiitis; EGPA, eosinophilic GPA; GN, glomerulonephritis; PAN, polyarteritis nodosa; cPAN, cutaneous PAN; TA, Takayasu’s arteritis; UPV, unclassifiable primary vasculitis; pVAS, pediatric vasculitis activity score. * Significant difference in median age between SVV and MVV (p = 0.0209), but not SVV and LVV (p = 0.0666) or MVV and LVV (p = 0.5357) based on Mann–Whitney tests.