Characteristics | Patient 1 | Patient 2 | Patient 3, Sibling of Patient 2 | Patient 4 | Patient 5 | Patient 6 |
---|---|---|---|---|---|---|
Age at onset, yrs | 6.5 | 8 | 3.5 | 4 | 9 | 10 |
Age at diagnosis, yrs | 13.5 | 10.5 | 15 | 22 | 22 | 21 |
Classification criteria for PAN* | Histopathology +2/5, cutaneous | Histopathology +3/5, systemic | Histopathology +angiography, +2/5, systemic | 5/10, systemic | 4/10, systemic | 4/10, systemic |
Previous treatment | NSAID, COL, MTX, CS | COL, MTX, CS, CYC, AZA, MMF, FFP | COL, CS, CYC, ETN | COL, CS, AZA, CYC, iloprost, FFP | CS, CYC, TCZ, IVIG, FFP | COL, CS, MTX, AZA |
Current treatment | MMF | ETN | FFP | ETN, plasmapheresis | — | COL |
Outcome | PVAS = 0 | PVAS = 0 | PVAS = 3 | BVAS = 25 | Died | BVAS = 0 |
↵* ACR 1990 criteria5 for adults and Ankara 2008 criteria6 for children. DADA2: deficiency of adenosine deaminase 2; PAN: polyarteritis nodosa; NSAID: nonsteroidal antiinflammatory drug; COL: colchicine; MTX: methotrexate; CS: corticosteroid; CYC: cyclophosphamide; AZA: azathioprine; MMF: mycophenolate mofetil; FFP: fresh frozen plasma; ETN: etanercept; TCZ: tocilizumab; IVIG: intravenous immunoglobulin; PVAS: pediatric vasculitis activity score; BVAS: Birmingham Vasculitis Activity Score; ACR: American College of Rheumatology.