Case of catastrophic antiphospholipid syndrome presenting as neuroretinitis and vaso-occlusive retinopathy
| Autor: | Young In Yun, Il-Soo Ha, Yo Han Ahn, Seon Hee Lim, Baek Lok Oh, Hee Gyung Kang, Ji Hyun Kim |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2020 |
| Předmět: |
medicine.medical_specialty
Thrombotic microangiopathy Adolescent genetic structures Case Report Catastrophic antiphospholipid syndrome Vaso-occlusive retinopathy Neuroretinitis 03 medical and health sciences 0302 clinical medicine lcsh:Ophthalmology Antiphospholipid syndrome Ophthalmology medicine Humans Glucocorticoids 030203 arthritis & rheumatology business.industry Microangiopathy Retinitis Acute kidney injury Warfarin General Medicine medicine.disease eye diseases Chorioretinitis lcsh:RE1-994 030221 ophthalmology & optometry Female Rituximab sense organs business Retinopathy medicine.drug |
| Zdroj: | BMC Ophthalmology, Vol 20, Iss 1, Pp 1-5 (2020) BMC Ophthalmology |
| ISSN: | 1471-2415 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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