Table 2.
Comparison of clinical features in PAN and DADA2 *
Adult PAN | Childhood PAN | DADA2 | |
---|---|---|---|
Average age at diagnosis (yr) | 50 | 9 | 5 |
Female (%) | 35 | 50 | 50 |
Clinical features | |||
Recurrent fever (%) | 60 | 80 | 70 |
Myalgia / arthralgia (%) | 60 | 70 | 50 |
Livedo (%) | 20 | 50 | 70 |
Cutaneous vasculitis (%) | 60 | 90 | 80 |
Neurologic manifestations (%) | 60 | 30 | 60 |
CNS involvement (%) | 10 | 20 | 60 |
PNS involvement (%) | 70 | 20 | 20 |
GI manifestations (%) | 30 | 40 | 40 |
Renal manifestations (%) | 40 | 40 | 20 |
Hypertension (%) | 40 | 30 | 20 |
Laboratory features ** | |||
Hemoglobin | ↓ | - | ↓ (50%) |
WBC | ↑ | - | ↓ (50%) |
Platelets | ↑ | - | ↓ (20%) |
IgA | - | - | ↓ (40%) |
IgM | - | - | ↓ (50%) |
IgG | - | - | ↓ (30%) |
Bold print highlights areas of major difference between DADA2 and PAN
Arrows indicate increased (↑) or decreased (↓) levels compared to healthy controls. The approximate percentage of DADA2 patients with each laboratory feature is provided.