Published in:
Open Access
01-12-2020 | Antiphospholipid Syndrome | Case report
Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy
Authors:
Young In Yun, Ji Hyun Kim, Seon Hee Lim, Yo Han Ahn, Hee Gyung Kang, Il-Soo Ha, Baek-Lok Oh
Published in:
BMC Ophthalmology
|
Issue 1/2020
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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.