Manifestações oculares da síndrome do anticorpo antifosfolipídio

Abstract

Antiphospholipid antibody syndrome (APS) is a rare, immune-mediated syndrome characterized by the presence of one or more episodes of arterial and/or venous and/or small vessel thrombosis and/or criteria of gestational morbidity, associated with the positivity of the antiphospholipid antibodies (aPL). The objectives of this study were to evaluate ocular manifestations in a cohort of patients with Primary Antiphospholipid Syndrome (PAS) and compare them with a healthy control group, to characterize the correlation of criteria and non-criteria manifestations with antiphospholipid antibodies (aPL) and to carry out a systematic review of literature. It was performed in two stages: in the first (first article), 105 patients were evaluated in relation to ophthalmological manifestations through retrospective analysis of medical records and by interview including ocular symptomatology, in addition to systematic review of the literature on ocular manifestations in patients with PAPS or with antiphospholipid antibodies (aPL). At this stage, we had an ophthalmological diagnosis in seven patients, with five occlusions of retinal vessels. Amaurosis fugax was the most common symptom, found in 30 patients, being related in the univariate analysis to Raynaud's phenomenon (p<0.048), anticardiolipin antibody (p<0.041) and hemianopsia related to arterial hypertension (p<0.049). In the multivariate analysis, an association between livedo reticularis and amaurosis fugax was found (p<0.006). In the systematic review, 96 articles were included, including anterior, posterior, orbital and neuro-ophthalmological findings. In the second stage (second article), we conducted a cross-sectional study with 98 patients with PAPS and 102 controls. Participants underwent anamnesis and ophthalmological evaluation. Eight-four PAPS patients had some ophthalmological manifestation (94.0% found in the posterior segment, 62.7% in the anterior segment and 56.6% had findings in both segments. Vascular tortuosity was more frequent in the SAFP group (63.2% vs. 42.2%; p=0.002), as well as peripheral tortuosity (29.6% vs. 7.8%; p<0.001). After excluding risk factors for atherosclerosis, peripheral tortuosity remained statistically associated with APS (35.0 vs. 7.8%, p<0.001). Furthermore, the triple positivity was related in PAPS patients with peripheral tortuosity and was not related in PAPS patients without this ocular finding (34.5% vs. 15.9%, p=0.041). Thus, due to the correlation with Raynaud's phenomenon and livedo, the first study suggests the probable vasomotor pathophysiology of amaurosis fugax in PAPS. In addition, the second study suggests that peripheral tortuosity may be a marker of ischemic retinopathy, being more present in the most severe serological phenotype (triple positives)