Table 1 (abstract P361). See text for description
From: Proceedings of the 28th European Paediatric Rheumatology Congress (PReS 2022)
Parameters | Our study, 2022 (n=20) | Lee, JS et al, 2021 (n=9) | Ozen et al, 2019 (n=66 Pediatric +67 Adult=133) | Eleftheriou et al, 2013 (n=69) |
|---|---|---|---|---|
Median age at diagnosis in years (Range in years) | 10 (4- 15) | 7.6 (3-17.5) | 16 (2-75) | 8.5 (0.9-15.8) |
Male: Female | 1:1 | 2:1 | 0.88:1 | 1.22:1 |
Clinical features: | ||||
 A. Fever | 95% | 77.7% | 75.7% | 87% |
B. Skin features: | ||||
 Purpura | 85% | 88.8% | NA | 41% |
 SC nodule | 55% | 33.3% | NA | 23% |
 Gangrene | 55% | 22.2% | 0 | 4% |
 Livedo reticularis | 15% | 22.2% | 54.5% | 49% |
 Ulcer | 25% | 22.2% | 4.5% | 4% |
 RP | 10% | 44.4% | 4.5% | 23% |
C. MSK features: | ||||
 Arthralgia/ Arthritis | 65% | 77.7% | 89.3% | 75% |
 Myalgia | 55% | 22.2% | 72.7% | 83% |
 D. Weight Loss | 45% | 11.1% | 30.3% | 64% |
E. GIT | Â | Â | 25.7% | Â |
 Abdomen pain | 20% | 22.2% | NA | 41% |
 Perforation/ischemia | 10% | 11.1% | NA | 4% |
F. Renal | Â | Â | 33.3% | Â |
 Proteinuria | 5% | NA | NA | 19% |
 Haematuria | 10% | NA | NA | 10% |
 Impaired function | 10% | 11.1% | NA | 15% |
G. Nervous system | Â | Â | 21.2% | Â |
 Stroke | 15% | 11.1% | NA | 10% |
 Peripheral neuropathy | 35% | 11.1% | NA | 8.69% |
 H. Testicular | 50% | NA | 6% | 10% |
 I. Hypertension | 15% | 11.1% | 39.3% | 16% |
Investigations: | ||||
 Skin biopsy | 70% | 88.8% | 94/133 | 72.4% |
 Angiography | 20% | 11.1% | 92/133 | 95.6% |
 ASLO | 30% |  |  |  |
Immunomodulator | ||||
 Corticosteroids | 100% | 100% | 133/133 | 100% |
 Cyclophosphamide | 60% | 55.5% | 62/133 | 86.9% |
 MMF | 40% | NA | 39/133 | 13% |
 AZA | 70% | 44.4% | 32/133 | 78% |
Diagnosis: | ||||
 Systemic PAN | 60% | 88.8% | 63/133 | 100% |
 Cutaneous PAN | 40% | 11.1% | 33/133 | 0 |
 Mortality | 10% | 0 | 0/66 | 4% |
 Relapses | 75% | 77.7% | 100% | 35% |
Median duration of follow up in years | 5.4 | 7 | 13 | 6 |