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P1-1
AUTOINFLAMMATORY CONDITIONS IN CHILDREN:
WHAT SHOULD WE KNOW?
Anne-Marie Prieur (Paris, France)
M a i n ly four groups of diseases are at risk of eye infl a m m ation with
va rious degrees of seve ri t y. 1) Ju venile Idiopathic A rt h ritis (JIA). Th e
most common is ch ronic anterior uveitis in the oligo a rticular fo rm
o c c u rring mostly in ve ry young gi rls and associated with serum antinu
clear antibody. Acute anterior uveitis can be observed is young boy s
with enthesitis associated art h ro p at hy. 2) Some autoimmune conditions
are ch a ra c t e ri zed by eye infl a m m ation. Wegener gra nu l o m at o s i s
ch a ra c t e ri zed by ANCA affects small and medium sized vessels with
s i nu s i t i s , upper re s p i rat o ry and pulmonary tra c t , k i d n ey… Eye manife
s t ations are from mild conjunctivitis to seve re infl a m m ation (ke rat i-
t i s , retinal vasculitis). Cogan syndrome consists of a ve s t i bu l o a u d i t o-
ry syndrome with sudden hearing loss and bilat e ral interstitial ke rat i-
tis. Sjögren syndro m e, a s s o c i ated to anti Ro anti La antibodies, i s
ch a ra c t e ri zed by dry eye with saliva ry glands swelling and dry mouth,
sometimes kidney invo l vement 3) Genetic disord e rs. The diseases
a s s o c i ated to mu t ations of the CIAS1, p a rt i c u l a rly CINCA, MWS are
a s s o c i ated with optic disk ch a n ge s , ( o e d e m a , at ro p hy) ch ronic anterior
uveitis and often visual loss. Meva l o n ate kinase defi c i e n cy due to
mu t ation in the MVK gene , also called hyperIgD syndrome is assoc
i ated to cat a racts in its most seve re type affecting the infant. Eye
i nvo l vement in Blau syndro m e, due to a mu t ation of the CARD15
gene (also called familial sarcoidosis) is part of the triad ch a ra c t e ri z-
ing the disease with skin rash and art h ritis. Eye invo l vement in Behçet
disease can affect anterior and/or posterior seg m e n t , or manifests as
i ri d o cy cl i t i s , hypopion or retinal va s c u l i t i s , optic disk hy p e remia and
macular oedema. 4) Other diseases such as tubu l o i n t e rstitial nep h ri t i s
and uveitis (TINU) or Vogt Koya n agi Harada syndrome are also assoc
i ated with eye manife s t at i o n s .
P1-2
EPIDEMIOLOGY OF CHILDHOOD UVEITIS: RESULTS
OF AN INTERNATIONAL RETROSPECTIVE MULTICEN-
TER ANALYSIS OF UVEITIS PATIENTS < 19 YEARS
Friederike Mackensen 1 , Sandra Prazeres 2 , Matthias D. Becker 1 ,
Marie-Sophie Enzmann 1 , Celine Terrada2, Bahram Bodaghi 2 ,
A n gela Wat k i n s 3 , G e o rge Reed 4 , Julie Fri e d l i n 5 , D i m i t ry
Pyatesky 5 , H. Nida Sen 4 , Debra Goldstein 5 , Justine R. Smith 3 ,
Janine A. Smith 4
1 Interd i s c i p l i n a ry Uveitis Center, U n ive rsity of Heidelberg,
Germany,
2 Hopital Pitie-Salpetriere, Paris, France,
3 Casey Eye Institute, OHSU, Portland, OR, USA,
4 National Eye Institute, Bethesda, MD, USA,
5University of Illinois, Chicago, IL, USA
P u rp o s e : To describe etiologies, disease activity, and prognosis of
pediatric uveitis.
M e t h o d s : Databases review from 5 uveitis centers (3 US and 2 EU)
with standardized definitions and time points.
R e s u l t s : 853 (527 US, 326 EU) patients with follow-up of 1-10 yrs
were identified. Median age at presentation was 11.2 (US) and 11.0
years (EU), median age at first diagnosis 9.1 (US) and 9 years
(EU). Both groups showed a similar gender distribution (F:M
1.2:1). Centers differed significantly in percentage of Caucasian
patients (EU 88%, US 51-78%).
The most frequent diagnosis in the US was idiopathic uveitis
(28.8%) followed by juvenile idiopathic arthritis (JIA) -related
uveitis (20.9%); in contrast JIA was the most frequent diagnosis in
EU centers (30.8%). In all centers JIA was more frequent among
the children with longer follow-up.
U veitis was active as judged by SUN cri t e ria in 75/79% of ch i l d re n
at the fi rst visit and in 55/40% at the 5 year visit (US/EU). Lega l
blindness (VA < 20/200 in the better eye) was diagnosed in 3/4% at
the fi rst visit and 9/10% at the 5 year and 8/12.5% at the 10 year visit.
Th e re was a negative correlation between VA > 20/50 and complications
at first visit, especially CME and hypotony (p = 0.006 and
0.026) in the US group and epiretinal membrane (p = 0.0425), band
keratopathy, CME and cataract (p = 0.05) in the EU cohort.
At first visit a mean number of 1.4/1.7 (US/EU) complications
were present per child: the most frequent complications were
cataract (28/22%), CME (16.6/11.6 %) and glaucoma (6.4/0.6 %).
In the group of children followed for at least 5 years, 56/46% had
cataract, 30.4/15.38% had CME, and 18.8/25% had glaucoma at
some time during this period.
95/91% received systemic immunosuppressive therapy at any time,
which involved combination therapy of prednisone (or equivalent)
and systemic immunosuppressive drugs (including biologics) in
37/37%. Use of systemic immu n o s u p p re s s ive drugs such as
methotrexate alone or in combination was more frequent in the EU
(52%) than in the US (27 %). The use of biologics was also slightly
more frequent in the EU (10%) than in the US (6%).
More children in EU (29%) than in the US (17%) had surgery during
the observed period. Cataract extraction was the most frequent
type of surgery.
C o n cl u s i o n : Children with uveitis followed by specialized centers
over a long period of time have active and severe disease which
re q u i res intensive tre at m e n t , f re q u e n t ly involving systemic
immunosuppressive drugs including biologics. Still only 10 -12.5%
of them showed legal blindness at the 5-10 year time point, a number
smaller than observed historically, that will hopefully further
decline with the increasing availability of a range of systemic
immunosuppressive drugs.
P1-3
CLINICAL CHARACTERISTICS
OF UVEITIS IN CHILDREN
Arellanes Lourdes
(Asociacion Para Evitar la Ceguera en México)
A review of the unique challenges that children with uveitis represents
will be made. Practical difficulties involved in getting an
accurate history and a complete eye examination will be analyzed.
Differences in clinical manifestations, uveitis syndromes and differential
diagnosis will be shown.
Medical therapy may also be more difficult because children with
uveitis have unique dosing requirements, drugs - associated risks
and compliance issues. In children a delayed uveitis diagnosis is
frequently made, which can be associated with development of
complications such as cataract, glaucoma, cyclitic membrane formation,
band keratopathy, etc. Great care must be taken when
choosing the best surgical technique in each case.
Financial fundings:None
Conflict of interest: None
Keywords: Different manifestations, compliance problems, different
diagnosis
9 th International Ocular Inflammation Society (IOIS) Congress
September 17-20, 2007, Paris, France
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