Fabrizia Corona

Federica Cuoco

Giancarla Di Landro

Giovanni Filocamo

Maria D'Errico

Sofia Torreggiani

Pediatric Rheumatology

PubMed

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Aortitis and uveitis. A challenging case of Takayasu or Behcet disease?

POSTER PRESENTATION Open Access
Aortitis and uveitis. A challenging case of
Takayasu or Behcet disease?
Giancarla Di Landro*, Federica Cuoco, Sofia Torreggiani, Maria Maddalena D’Errico, Giovanni Filocamo,
Fabrizia Corona
From 21st European Pediatric Rheumatology (PReS) Congress
Belgrade, Serbia. 17-21 September 2014
Introduction
Childhood vasculitis is a group of conditions that are
defined as the presence of blood vessel inflammation,
and they are grouped in base of the size of vessels
involved. In 2008 were presented the PRES revised clas-
sification criteria for childhood vasculitis.
Objectives
We describe the case of a vasculitis interesting the aortic
arch in a boy with panuveitis, HLA B51 positive and
proteinuria.
Methods
We presented a challenging case of vasculitis classified
considering the EULAR/PRINTO/PRES c-Takayasu
Arteritis criteria of 2008 and the International Criteria
for Behcet disease of 2013 (ICBD)
Results
B. is a ten years old boy born in Columbia. At the age of
nine, he presented fever that lasted for about 1 month.
In the suspect of Kawasaki disease, he was administered
Immunoglobulin, without resolution, that persisted until
administration of intravenous steroid. His clinical condi-
tions were characterized by asthenia, arthralgia and
photophobia. He also presented a heart murmur of 2/6
at centrum cordis. He presented high inflammatory
markers, HLA-B51 positivity and proteinuria. Chest
radiography, abdomen ultrasound, brain MRI, lumbar
puncture and cardiac ultrasound were negative. Instead
total body PET and MRI showed inflammation of aortic
arch and signs of previous pericarditis. The eye exami-
nation showed panuveitis with retinitis. The therapy
administered was based on sistemic and ocular steroids
and on mycophenolate mofetil with benefit. Table 1.
UOS Reumatologia Pediatrica, Pediatria a Media Intensità di Cura,
Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Milan, Italy
Table 1 Clinical features of our patient and differential diagnosis based on PRES c-TA and BD criteria
EULAR/PRINTO/PRES c-TA criteria Our patient ICBD-point score system Sign/symptom Score Our patient
Angiographic abnormality Yes Ocular lesion 2 Yes
1. Pulse deficit or claudication Not Genital aphthosis 2 Not
2. Blood pressure discrepancy Not Oral aphthosis 2 Not
3. Bruits Not Skin lesions 1 Doubtful
4. Hypertension Not Neurological manifestations 1 Not
5. Acute phase reactant Yes Vascular manifestation 1 yes
Positive pathergy test 1 not performed
Di Landro et al. Pediatric Rheumatology 2014, 12(Suppl 1):P354
http://www.ped-rheum.com/content/12/S1/P354
© 2014 Di Landro et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative
Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and
reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver
(http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
Conclusion
Making the diagnosis of vasculitis is often challenging,
because presenting symptoms may be subacute, non-
specific and non diagnostic. Our patient had clinical
manifestations, signs and symptoms of TA and BD. The
TA criteria were satisfied by the presence of the aorta
thickening and high inflammatory markers, however clin-
ical features such as pericarditis, uveitis and HLA-B51
positivity are suggestive for BD, even if the BD criteria
are not fulfilled. Vasculitis classifications are useful for
patient categorization however often other clinical char-
acteristics must be considered in distinguishing ambigu-
ous situations.
Disclosure of interest
None declared.
Published: 17 September 2014
doi:10.1186/1546-0096-12-S1-P354
Cite this article as: Di Landro et al.: Aortitis and uveitis. A challenging
case of Takayasu or Behcet disease? Pediatric Rheumatology 2014 12
(Suppl 1):P354.
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Di Landro et al. Pediatric Rheumatology 2014, 12(Suppl 1):P354
http://www.ped-rheum.com/content/12/S1/P354
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Aortitis and uveitis. A challenging case of Takayasu or Behcet disease?

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