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Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy

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Authors
Yun, Young In; Kim, Ji Hyun; Lim, Seon Hee; Ahn, Yo Han; Kang, Hee Gyung; Ha, Il-Soo; Oh, Baek-Lok
Issue Date
2020-12-09
Publisher
BMC
Citation
BMC Ophthalmology. 2020 Dec 09;20(1):482
Keywords
Catastrophic antiphospholipid syndromeAntiphospholipid syndromeNeuroretinitisVaso-occlusive retinopathyThrombotic microangiopathy
Abstract
Background
Ocular involvement in catastrophic antiphospholipid syndrome (CAPS), a rare, life-threatening form of antiphospholipid syndrome (APS) that results in multiorgan failure and a high mortality rate, has rarely been reported.

Case presentation
A 15-year-old girl presented with sudden vision blurring in both eyes. She had marked optic disc swelling and macular exudates in the right eye and intra-arterial white plaques, a few retinal blot hemorrhages, and a white ischemic retina in the left eye. Systemic examination revealed she had acute kidney injury with thrombotic microangiopathy (TMA), multiple cerebral infarcts, valvular dysfunction, and a high titer of triple aPL. Thus, she was diagnosed with CAPS involving the brain, eyes, heart, and kidneys. Plasma exchange and the administration of glucocorticoids, immunoglobulin, warfarin, and rituximab brought a sustained recovery of the TMA, visual symptoms, and echocardiographic findings.

Conclusions
Ocular involvement of both vaso-occlusive retinopathy, an APS-related thrombotic microangiopathy, and neuroretinitis, a non-thrombotic microangiopathy, can occur as an initial presentation of CAPS.
ISSN
1471-2415
Language
English
URI
https://hdl.handle.net/10371/173406
DOI
https://doi.org/10.1186/s12886-020-01755-9
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College of Medicine/School of Medicine (의과대학/대학원)Ophthalmology (안과학전공)Journal Papers (저널논문_안과학전공)
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