Page 174 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

150 Ezgi Deniz Batu and Seza Ozen

criterion was deleted, and the “angiographic abnormalities” was made a mandatory criterion
and modified with imaging modalities such as CT and magnetic resonance imaging (MRI)
[24]. Hypertension which may be the only symptom in children with TAK at presentation was
included as a new criterion [22].

An increase in acute phase reactant levels was included in the criteria as this was an
important finding before the onset of complications and, one that could assist in differential
diagnosis. The final classification criteria for cTAK included the mandatory criteria of
angiographic abnormalities of the aorta and its main branches, and pulmonary arteries
showing aneurysms and dilatations, plus one of the five following features including, pulse
deficit or claudication, four-limb blood pressure discrepancy, bruits, hypertension, and
elevation of acute phase reactants [24]. With all the incorporated modifications, the
classification of cTAK criteria achieved a sensitivity of 100% and a specificity of 99% [24].

Conclusion

Primary systemic vasculitides are relatively uncommon in children in comparison to
adults. Certain vasculitides such as KD occur almost exclusively in childhood and although
both children and adults share many common characteristics in vasculitis, they can differ
substantially in certain aspects such as the etiology, frequency of clinical features, ultimate
prognosis and response to therapy. As most of the pediatric vasculitides are rare diseases,
collaborative multicenter studies are needed to improve these criteria while advancing in
methods of diagnosis and prognostication.

References

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