Al-Obadi, Muthana

Maritsi, Despoina

Melo-Gomes, Sonia

Pilkington, Clarissa A

Tullus, Kjell

English

PubMed

Pilkington Clarissa A

Tullus Kjell

Melo-Gomes Sonia

Al-Obadi Muthana

Maritsi Despoina

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Pediatric Rheumatology

Systemic Lupus Erythematosus complicated by Neuromyelitis Optica (Devic’s Syndrome): case series from a single paediatric rheumatology centre

Despoina Maritsi

Muthana Al-Obadi

Sonia Melo-Gomes

Kjell Tullus

Clarissa A Pilkington

Springer - Publisher Connector

POSTER PRESENTATION Open Access
Systemic Lupus Erythematosus complicated by
Neuromyelitis Optica (Devic’s Syndrome): case
series from a single paediatric rheumatology
centre
Despoina Maritsi1*, Muthana Al-Obadi1, Sonia Melo-Gomes1, Kjell Tullus2, Clarissa A Pilkington1
From 18th Pediatric Rheumatology European Society (PReS) Congress
Bruges, Belgium. 14-18 September 2011
Objective
To identify the incidence of Neuromyelitis Optica
(NMO) in patients with paediatric Systemic Lupus
Erythematosus (SLE) and describe its pattern of
presentation.
Background
NMO or Devic’s syndrome is a rare autoimmune
demyelinating disease of the central nervous system
manifesting with transverse meylitis involving three or
more continuous segments and optic neuritis in the pre-
sence of NMO IgG antibodies.
Methods
Retrospective case study of all SLE patients with CNS
symptoms, diagnosed from 2000-2010 and review of the
clinical data, laboratory and MRI findings. Data were
collected from the medical records and were analyzed
using SPSS 2010.
Setting: A tertiary referral centre for juvenile SLE.
Results
A total of 210 (161 females, 49 males) SLE patients were
indentified, 39 of which had manifestations of potential
CNS involvement and underwent imaging of their CNS
including spinal cord. Three were indentified with prob-
able Devic syndrome, which was confirmed in two
(0.9%). Both patients were adolescent females and of
Caucasian origin. In one patient NMO was the first
manifestation of SLE. In the other NMO developed
three years following diagnosis of SLE. They both pre-
sented with deterioration of visual acuity, localized spine
tenderness and malaise. NMO was confirmed based on
MRI findings and the presence of raised Aquaporin-4
IgG antibodies in the plasma. On both occasions NMO
had a relapsing course and interestingly NMO relapses
coincided with SLE disease flare-up, which responded to
treatment simultaneously. The patient with ongoing SLE
had a more severe course and required more intensive
immunosuppressive treatment. Both patients developed
depression following diagnosis of NMO.
Discussion
SLE is a multisystemic autoimmune disease and 25% of
the patients will develop CNS involvement throughout
its course. While NMO has been described in adult
patients with SLE, these cases derive specifically from a
paediatric population. We believe that SLE and NMO
are parts of the same disease spectrum. When this con-
dition is noticed in patients with refractory, long stand-
ing SLE, prognosis is guarded. The burden of chronic
illness on mental health status and on deterioration of
quality of life is well documented in adults. However,
physicians working with paediatric patients should be
reminded of the psychological component that needs to
be addressed in each visit.
Author details
1Department of Paediatric and Adolescent Rheumatology, Great Ormond
Street Hospital for Children NHS Trust, London, UK. 2Department of
1Department of Paediatric and Adolescent Rheumatology, Great Ormond
Street Hospital for Children NHS Trust, London, UK
Full list of author information is available at the end of the article
Maritsi et al. Pediatric Rheumatology 2011, 9(Suppl 1):P241
http://www.ped-rheum.com/content/9/S1/P241
© 2011 Maritsi 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/2.0), which permits unrestricted use, distribution, and reproduction in
any medium, provided the original work is properly cited.
Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Trust,
London, UK.
Published: 14 September 2011
doi:10.1186/1546-0096-9-S1-P241
Cite this article as: Maritsi et al.: Systemic Lupus Erythematosus
complicated by Neuromyelitis Optica (Devic’s Syndrome): case series
from a single paediatric rheumatology centre. Pediatric Rheumatology
2011 9(Suppl 1):P241.
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Maritsi et al. Pediatric Rheumatology 2011, 9(Suppl 1):P241
http://www.ped-rheum.com/content/9/S1/P241
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Systemic Lupus Erythematosus complicated by Neuromyelitis Optica (Devic’s Syndrome): case series from a single paediatric rheumatology centre

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