Benseler et al identified factors associated with disease progression in a child with primary angiitis of the central nervous system (childhood PACNS, or cPACNS). These can help distinguish this high risk group from those with nonprogressive disease. The authors are from the Hospital for Sick Children in Toronto and University Children's Hospital in Bonn.
The nonprogressive group has a monophasic vasculitis with vasospasm that is rapidly reversible.
Progression was defined on angiography performed > 3 months after the initial diagnosis.
Risk factors associated with disease progression:
(1) neurocognitive dysfunction (likelihood ratio 40)
(2) multifocal parenchymal lesions on MRI (odds ratio 3)
(3) distal stenoses on angiography (odds ratio 15)
The authors combined these risk factors into a profile. This profile is only partially described in the paper. Based on the available material, the following profile table is proposed.
Factors Present |
Risk Score |
Risk of Disease Progression |
none |
1 |
very low |
multifocal lesions |
2 |
low |
distal stenoses |
3 |
low to moderate |
neurocognitive defects |
4 |
moderate |
multifocal lesions and distal stenoses |
5 |
moderate |
multifocal lesions and neurocognitive defects |
6 |
moderate to high |
distal stenoses and neurocognitive defect |
7 |
moderate to high |
all 3 |
8 |
high |
Purpose: To identify a child with primary angiitis of the central nervous system (cPACNS) who is likely to progress using the profile of Benseler et al.
Specialty: Immunology/Rheumatology
Objective: risk factors, selection, disease progression
ICD-10: I77.6,