2022
DOI: 10.1016/j.ajoc.2022.101266
|View full text |Cite
|
Sign up to set email alerts
|

Abstract: Purpose Autoimmune Polyendocrinopathy Syndrome (APS) is a rare condition caused by an autoimmune failure of two or more endocrine glands. In this case, we report the ocular findings and correlated histopathology from a human eye donor with a prior clinical history of Type 1 APS. Observations The 23 year-old patient originally presented with blurred vision at the 20/125 level caused by papilledema of the right eye. Bilateral pigmentary changes in the peripheral retinal w… Show more

Help me understand this report

Search citation statements

Order By: Relevance

Paper Sections

Select...
1

Citation Types

0
2
0

Year Published

2022
2022
2023
2023

Publication Types

Select...
4

Relationship

0
4

Authors

Journals

citations
Cited by 4 publications
(2 citation statements)
references
References 12 publications
0
2
0
Order By: Relevance
“…Clinical posterior segment findings associated with APECED include peripheral pigmentary changes, macular atrophy and constricted visual fields. On optical coherence tomography, disruption of external limiting membrane and inner segment ellipsoid band, and on fundus autofluorescence, features like parafoveal hyperautofluorescent ring and stippled patchy macular autofluorescence resembling retinitis pigmentosa have been described 3 9 10…”
Section: Discussionmentioning
confidence: 99%
“…Clinical posterior segment findings associated with APECED include peripheral pigmentary changes, macular atrophy and constricted visual fields. On optical coherence tomography, disruption of external limiting membrane and inner segment ellipsoid band, and on fundus autofluorescence, features like parafoveal hyperautofluorescent ring and stippled patchy macular autofluorescence resembling retinitis pigmentosa have been described 3 9 10…”
Section: Discussionmentioning
confidence: 99%
“…The deficiency of AIRE impairs central immune tolerance, generating pathogenic autoreactive T cells and autoantibodies directed against many specific cytokines, such as IL-17 and IL-22 [ 6 ]. Pigmented retinitis is considered as one of the APECED manifestations, affecting 3.6% of APECED patients [ 7 , 8 ]. It destroys the visual field in the early stages and affects vision, leading to blindness in the later stages [ 9 , 10 ].…”
mentioning
confidence: 99%