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

• Published in: BMC ophthalmology Vol. 20; no. 1; p. 482
• Main Authors: In Yun, Young, Kim, Ji Hyun, Lim, Seon Hee, Ahn, Yo Han, Kang, Hee Gyung, Ha, Il-Soo, Oh, Baek-Lok
• Format: Journal Article
• Language: English
• Published: England BioMed Central Ltd 09.12.2020; BioMed Central; BMC
• Subjects: Adolescent; Aneurysms; Antibodies; Anticoagulants; Antiphospholipid syndrome; Antiphospholipid Syndrome - complications; Antiphospholipid Syndrome - diagnosis; Autoimmune diseases; Blood pressure; Case Report; Case reports; Catastrophic antiphospholipid syndrome; Chorioretinitis; Corticosteroids; Edema; Exudates; Eye; Female; Glucocorticoids; Glycoproteins; Health aspects; Humans; Hypertension; Immunotherapy; Ischemia; Kidneys; Laboratories; Lupus; Medical prognosis; Monoclonal antibodies; Mortality; Neuroretinitis; Pathophysiology; Patients; Plaques; Retina; Retinitis; Retinitis - diagnosis; Retinitis - etiology; Retinopathy; Rituximab; Teenage girls; Thrombocytopenia; Thrombosis; Thrombotic microangiopathy; Vaso-occlusive retinopathy; Warfarin; Thrombotic microangiopathy; Neuroretinitis; Catastrophic antiphospholipid syndrome; Vaso-occlusive retinopathy; Antiphospholipid syndrome
• ISSN: 1471-2415; 1471-2415
• DOI: 10.1186/s12886-020-01755-9

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. 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. 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.