Page 238 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Polyarteritis Nodosa 213

more favorable for children than adults with an overall mortality rate of 1% to 16%. Relapses
are more frequent and occur only many years following diagnosis, sometimes preceded by
ear, nose and throat infections. Penicillin can contribute to a cure by effectively lowering the
relapse rate when Streptococci are involved as the cause or target factor [77]; however the
optimal antibiotic regimen is unknown. Relapses have been described up to 20 years after the
first episode.

Table 4. EULAR/PReS Proposed Classification Criteria for
Childhood Polyarteritis Nodosa*

A systemic illness characterized by the presence of at least 2 of the following 7 criteria:
1. Skin involvement (livedo reticularis, tender subcutaneous nodules, other vasculitic lesions)
A systemic illness characterized by the presence of at least 2 of the following 7 criteria:
2. Myalgia or muscle tenderness
3. Systemic hypertension, relative to normal childhood values
4. Mononeuropathy or polyneuropathy
5. Abnormal urine analysis and/or impaired renal function
6. Testicular pain or tenderness
7. Signs or symptoms suggesting vasculitis of any other major organ system (GI, cardiac,
respiratory or CNS)
In the presence of one, at least, of the following as a mandatory criterion
1. Biopsy showing small- and medium-sized artery necrotizing vasculitis
2. Angiographic abnormalities† (aneurysms or occlusions)
*Adapted from reference [75]. †Should include angiography if MR angiography is negative.

Abbreviations: EULAR/PReS, European League against Rheumatism/Pediatric Rheumatology
European Society; GI, gastrointestinal; CNS, central nervous system.

Laboratory Evaluation

Inflammation markers are found in the majority of patients. A one hour erythrocyte
sedimentation rate (ESR) >60 mm is noted in three-quarters of patients; and an elevated C-
reactive protein, ?-2 globulin levels and white blood-cell counts in one-half to three-quarters
of patients. Eosinophilia >1,500/mm3 and normochromic anemia can occur. HBsAg and
ANCA serology should be routinely tested.

Angiography

Bron and coworkers (78) showed the diagnostic value of angiography in visualizing
saccular or fusiform microaneurysms measuring 1 to 5 mm in diameter, and stenosis in
medium-sized vessels. Although not pathognomonic for PAN, they are predominantly seen in
the kidneys, mesentery and liver. Angiography is a useful tool when other diagnostic
examinations are negative, especially when abdominal pain and nephropathy are present.

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