Table 1.
Clinical characteristics at diagnosis.
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | Patient 6 | Patient 7 | Patient 8 | Patient 9 | |
|---|---|---|---|---|---|---|---|---|---|
| Sex | Male | Male | Male | Female | Male | Male | Male | Female | Female |
| Subtype | Systemic | Systemic | Systemic | Systemic | Systemic | Systemic | Systemic | Cutaneous | Systemic |
| Age at diagnosis (years) | 17.6 | 5.8 | 5.6 | 3.5 | 7.5 | 9.4 | 7.7 | 15.3 | 11.1 |
| Diagnosis delay (days) | 30 | 30 | 62 | 192 | 31 | 89 | 41 | 249 | 32 |
| Initial symptoms at diagnosis | |||||||||
| General | |||||||||
| Fevera | + | + | + | + | + | + | + | − | − |
| Weight lossb | + | − | − | − | − | − | − | − | − |
| Arthralgia | + | + | + | − | + | + | + | − | + |
| Myalgia | − | − | − | − | − | − | + | − | + |
| Skin | |||||||||
| Purpura | + | + | − | + | + | + | + | + | + |
| Subcutaneous nodules | + | − | + | − | − | − | − | − | + |
| Livedo reticularis | − | + | − | + | − | − | − | − | − |
| Skin infarct | − | − | − | − | − | − | + | − | + |
| Raynaud’s phenomenon | − | − | − | − | + | + | + | − | + |
| Gangrene | − | − | − | − | + | + | − | − | − |
| Nervous system | |||||||||
| Ptosis | − | − | − | + | − | − | − | − | − |
| Gait disturbance | − | − | − | + | − | − | − | − | − |
| Headache | − | − | − | + | − | − | − | − | − |
| Stroke | − | − | − | + | − | − | − | − | − |
| Renal | |||||||||
| Hypertension | − | − | − | + | − | − | − | − | − |
| Serum Cr↑c | − | − | − | + | − | − | − | − | − |
| Abdominal | |||||||||
| Abdominal pain | − | − | − | + | − | + | − | − | − |
| Bowel ischemia | − | − | − | + | − | − | − | − | − |
| Initial diagnosis | EN, Reactive arthropathy | Synovitis r/o JIA | Septic arthritis r/o vasculitis | Multiple sclerosis | Unspecified vasculitis | Unspecified vasculitis | Synovitis r/o JIA | EN | Psoriatic arthritis |
| FANA | − | − | − | − | − | − | − | 1:320 | − |
| ANCA | − | − | − | − | − | − | − | − | − |
| Other autoantibodies | Rheumatoid factor | ||||||||
| Steroid Hxd | + | − | + | + | + | + | + | + | + |
| Diagnostic method | Skin biopsy | Skin biopsy | Skin biopsy | Angiography | Skin biopsy | Skin biopsy | Skin biopsy | Skin biopsy | Skin biopsy |
| FSS | 0 | 0 | 0 | 2 | 0 | 0 | 0 | 0 | 0 |
| PVAS | 6 | 6 | 4 | 32 | 9 | 13 | 7 | 2 | 7 |
EN: erythema nodosum; JIA: juvenile idiopathic arthritis; FANA: antinuclear antibody; ANCA: anti-neutrophil cytoplasmic antibody; FFS: Five-Factor Score; PVAS: Paediatric Vasculitis Activity Score.
aDocumented axillary temperature, raised threshold to 38.0 °C.
bLoss of dry body weight without dieting ≥ 2 kg.
cIncrease in serum creatinine > 30%, from 0.4 to 0.6 mg/dL.
dHistory, treated with prednisolone or methylprednisolone pulse therapy prior to the diagnosis of childhood-onset PAN.