Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Dec 9;20(1):482.
doi: 10.1186/s12886-020-01755-9.

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

Young In Yun  1 , Ji Hyun Kim  2 , Seon Hee Lim  3 , Yo Han Ahn  3 , Hee Gyung Kang  3 , Il-Soo Ha  3 , Baek-Lok Oh  4
Affiliations
Case Reports

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

Young In Yun et al. BMC Ophthalmol. .

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.

Keywords: Antiphospholipid syndrome; Catastrophic antiphospholipid syndrome; Neuroretinitis; Thrombotic microangiopathy; Vaso-occlusive retinopathy.

PubMed Disclaimer

Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Ocular manifestations of a pediatric catastrophic anti-phospholipid syndrome. a Severe optic disc swelling, multiple blot hemorrhage, and macular exudates are seen in the right eye. b Optic disc swelling, macular exudates, multiple hemorrhages, intraarterial white plaques and white ischemic retina are observed in the left eye. c, d, Fluorescein angiographies revealed disc and vascular leakage with decreased choroidal perfusion are found in both eyes, and large non-perfusion area at temporal retina in the left eye. e, f, g, h Optical coherence tomography showed fluid and multiple hard exudates in subretinal space and Henle’s layer at macula and peripapillary area. Bilateral optic disc edema is observed
Fig. 2
Fig. 2
Ocular findings of pediatric catastrophic anti-phospholipid syndrome at 6 months after presentation. a The fundus showed no optic disc edema, but a few remnant exudates are seen in the right eye. b Occluded retinal arteries and laser marks at temporal retinal area are observed in the left eye. c, d Fluorescein angiographies revealed no disc leakage in the both eyes. Although there is non-perfused retinal area, no active retinal neovascularization is observed. e, f Optical coherence tomography of macula showed flat macula with a few remnant exudates are detected in the both eyes. Temporal retinal thinning is observed in the left eye. g Nasal field defect is shown in the left eye. h No definite visual field defect is detected in the right eye
See this image and copyright information in PMC

References

    1. Cervera R, Rodríguez-Pintó I, Colafrancesco S, Conti F, Valesini G, et al. 14th international congress on antiphospholipid antibodies task force report on catastrophic antiphospholipid syndrome. Autoimmun Rev. 2014;13(7):699–707. doi: 10.1016/j.autrev.2014.03.002. - DOI - PubMed
    1. Rodríguez-Pintó I, Moitinho M, Santacreu I, Shoenfeld Y, Erkan D, et al. Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of 500 patients from the international CAPS registry. Autoimmun Rev. 2016;15(12):1120–1124. doi: 10.1016/j.autrev.2016.09.010. - DOI - PubMed
    1. Farooqui SZ, Thong BY, Teoh SC. Neuroretinitis as an initial presentation of lupus-like illness with antiphospholipid syndrome. Lupus. 2010;19(14):1662–1664. doi: 10.1177/0961203310377220. - DOI - PubMed
    1. Kurz DE, Wang RC, Kurz PA. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis in a patient with antiphospholipid syndrome. Arch Ophthalmol. 2012;130(2):257–258. doi: 10.1001/archopthalmol.2011.1363. - DOI - PubMed
    1. Moosavi M, Hosseini SM, Shoeibi N, Ansari-Astaneh M-R. Unilateral idiopathic retinal vasculitis, aneurysms, and neuroretinitis syndrome (IRVAN) in a young female. J Curr Ophthalmol. 2015;27(1–2):63–66. doi: 10.1016/j.joco.2015.10.006. - DOI - PMC - PubMed

Publication types

LinkOut - more resources