Table 1.

Diagnostic criteria for PAN in adults and children

1990 ACR criteria for the classification of polyarteritis nodosa [37]*

1) Weight loss of 4 kg or more

2) Livedo reticularis

3) Testicular pain/tenderness

4) Myalgia or leg weakness/tenderness

5) Mononeuropathy or polyneuropathy

6) Diastolic blood pressure greater than 90 mm/Hg

7) Elevated blood urea nitrogen or creatinine level unrelated to dehydration or obstruction

8) Presence of hepatitis B surface antigen or antibody in serum

9) Arteriogram demonstrating aneurysms or occlusions of the visceral arteries

10) Presence of polymorphonuclear neutrophils in a biopsy specimen from a small- or medium-sized artery

2008 EULAR/PRINTO/PRES childhood PAN criteria [17, 18]

A systemic illness characterized by the presence of either a biopsy showing small and medium-size artery necrotizing vasculitis

OR

Angiographic abnormalities demonstrating aneurysms or occlusions

plus at least two of the following:

1) Skin involvement (livedo reticularis, subcutaneous nodules, ulcerations and necrosis)

2) Myalgia or muscle tenderness

3) Hypertension (> 95% for height)

4) Peripheral neuropathy

5) Renal involvement

^*presence of 3 or more criteria is considered diagnostic for PAN