Characterization of visual manifestations and identification of risk factors for permanent vision loss in patients with giant cell arteritis.

BACKGROUND/PURPOSE: Permanent vision loss (PVL) is a feared complication and a leading cause of morbidity in giant cell arteritis (GCA). The objective of this study is to describe visual manifestations and identify risk factors of ocular involvement in GCA. METHODS: A retrospective database from a single vasculitis referral center was used. Descriptive statistics comparing patients with and without ocular involvement were performed. RESULTS: One hundred patients with GCA were included. Visual symptoms were present in 53% of patients at diagnosis and included blurred vision (30%), diplopia (16%), amaurosis fugax (14%), and blindness (19%). Out of 19 patients with blindness, 16 did not recover and had PVL. Patients with PVL were older (79.2 ± 6.7 vs 74.2 ± 7.6 years; p = 0.008) and more likely to have coronary artery disease (31% vs 10%; p = 0.018). However, they were less likely to have other cranial symptoms (81% vs 96%; p = 0.019), mainly headaches (64% vs 92%; p = 0.003). Risk factors associated with an abnormal ophthalmologic examination were the same as for PVL, but patients were also more likely to have diabetes (29% vs 7%; p = 0.040) and less likely to have constitutional symptoms (53% vs 80%; p = 0.033). CONCLUSION: Patients with GCA and ocular involvement were more likely to have baseline diabetes and atherosclerosis. A predisposing vascular vulnerability might therefore increase the risk of ocular involvement. Key points • Most patients with GCA and complete vision loss at presentation will not recover and evolve to have permanent vision loss. • A GCA patient with visual manifestations at presentation has more baseline vascular risk factors (diabetes, atherosclerosis) than patients without ocular involvement. • Patients with GCA and visual manifestations have fewer constitutional symptoms and lower inflammatory markers than patients without ocular involvement.

Continuous Renal Replacement Therapy with oXiris® Membrane in Severe Ebstein-Barr Virus-Mediated Hemophagocytic Lymphohistiocytosis: A Case Report.

Hemophagocytic lymphohistiocytosis (HLH), a life-threatening disease with uncontrolled immune activation and inflammatory reaction, often leads to a deadly cytokine storm. In severe Ebstein-Barr virus-triggered HLH receiving standard immunosuppression, continuous renal replacement therapy (CRRT) with oXiris® blood purification membrane resulted in a timely reduction of inflammatory markers and discontinuation of vasopressors. To our knowledge, this is the first report of successful use of the oXiris® membrane in HLH.