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Manifestation of panuveitis after intraocular surgery in a child with blau syndrome

Magliyah, Moustafa ; Khan, Abdullah ; Alshamrani, Mohammed ; Schatz, Patrik LU orcid and Dhibi, Hassan (2021) In Middle East African Journal of Ophthalmology 28(3). p.196-198
Abstract

Blau syndrome (BS) is a rare granulomatous disease with autosomal dominant inheritance. It is characterized by a triad of dermatitis, arthritis, and recurrent uveitis. This case presents the onset of panuveitis in BS after intraocular surgery. A 10-year-old boy presented to the outpatient clinic with retinal detachment in the left eye after 6 years following early-onset cataract surgery. Bilateral panuveitis occurred 3 weeks after surgical repair and resulted in a total visual loss in the left eye and was persistent to conventional treatment in the right eye. Genetic testing revealed a mutation in NOD2 gene. The addition of adalimumab to the treatment regimen resulted in long-term uveitis control and maintenance of 20/70 vision in the... (More)

Blau syndrome (BS) is a rare granulomatous disease with autosomal dominant inheritance. It is characterized by a triad of dermatitis, arthritis, and recurrent uveitis. This case presents the onset of panuveitis in BS after intraocular surgery. A 10-year-old boy presented to the outpatient clinic with retinal detachment in the left eye after 6 years following early-onset cataract surgery. Bilateral panuveitis occurred 3 weeks after surgical repair and resulted in a total visual loss in the left eye and was persistent to conventional treatment in the right eye. Genetic testing revealed a mutation in NOD2 gene. The addition of adalimumab to the treatment regimen resulted in long-term uveitis control and maintenance of 20/70 vision in the right eye. We propose that NOD2-mediated inflammatory cascade can be activated by intraocular surgery and results in the manifestation of BS.

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author
; ; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Blau syndrome, intraocular surgery, NOD2, panuveitis
in
Middle East African Journal of Ophthalmology
volume
28
issue
3
pages
3 pages
publisher
Wolters Kluwer
external identifiers
  • pmid:35125805
  • scopus:85122648080
ISSN
0974-9233
DOI
10.4103/meajo.meajo_15_21
language
English
LU publication?
yes
id
a0cec052-d2a4-46d4-84e6-d88583464a77
date added to LUP
2022-03-08 14:32:57
date last changed
2024-03-31 01:09:47
@article{a0cec052-d2a4-46d4-84e6-d88583464a77,
  abstract     = {{<p>Blau syndrome (BS) is a rare granulomatous disease with autosomal dominant inheritance. It is characterized by a triad of dermatitis, arthritis, and recurrent uveitis. This case presents the onset of panuveitis in BS after intraocular surgery. A 10-year-old boy presented to the outpatient clinic with retinal detachment in the left eye after 6 years following early-onset cataract surgery. Bilateral panuveitis occurred 3 weeks after surgical repair and resulted in a total visual loss in the left eye and was persistent to conventional treatment in the right eye. Genetic testing revealed a mutation in NOD2 gene. The addition of adalimumab to the treatment regimen resulted in long-term uveitis control and maintenance of 20/70 vision in the right eye. We propose that NOD2-mediated inflammatory cascade can be activated by intraocular surgery and results in the manifestation of BS.</p>}},
  author       = {{Magliyah, Moustafa and Khan, Abdullah and Alshamrani, Mohammed and Schatz, Patrik and Dhibi, Hassan}},
  issn         = {{0974-9233}},
  keywords     = {{Blau syndrome; intraocular surgery; NOD2; panuveitis}},
  language     = {{eng}},
  number       = {{3}},
  pages        = {{196--198}},
  publisher    = {{Wolters Kluwer}},
  series       = {{Middle East African Journal of Ophthalmology}},
  title        = {{Manifestation of panuveitis after intraocular surgery in a child with blau syndrome}},
  url          = {{http://dx.doi.org/10.4103/meajo.meajo_15_21}},
  doi          = {{10.4103/meajo.meajo_15_21}},
  volume       = {{28}},
  year         = {{2021}},
}