Ocular involvement in primary antiphospholipid syndrome: results of an extensive ophthalmological evaluation performed in the APS-Rio cohort.

OBJECTIVE: To study ophthalmological manifestations in a well-characterized primary antiphospholipid syndrome (PAPS) cohort (APS-Rio) and compare them with a healthy control group. METHODS: We examined PAPS patients and controls with an extensive ophthalmological evaluation, which included anamnesis, visual acuity, slit-lamp biomicroscopy, binocular indirect ophthalmoscopy, and retinography of the anterior and posterior segments of the eye. PAPS group also underwent angiography exam and optical coherence tomography using spectral domain technology (SD-OCT). RESULTS: 98 PAPS patients and 102 controls were included. The most common symptom in PAPS was amaurosis fugax (34.7% vs. 6.9%; p = .001). In the multivariate analyses, Raynaud's phenomenon was associated with amaurosis fugax (OR 3.71, CI:1.33-10.32; p = .012), and livedo correlated with hemianopia (OR 6.96, CI:1.11-43.72, p = .038) and diplopia (OR 3.49, CI:1.02-11.53, p = .047). After ophthalmological evaluation, 84 PAPS patients had ocular involvement (1.0% glaucoma, 94.0% posterior findings, 62.7% anterior findings, and 56.6% both posterior and anterior findings). Vascular tortuosity was more frequent in the PAPS group (63.2% vs. 42.2%; p = .002), as well as peripheral tortuosity (29.6% vs. 7.8%; p < .001). After excluding patients with atherosclerotic risk factors, peripheral vascular tortuosity was still statistically associated with PAPS (35.0 vs. 7.8%, p < .001). Triple positivity was more frequent in PAPS patients with peripheral vascular tortuosity than in those without this ocular finding (34.5% vs. 15.9%, p = .041). CONCLUSION: Vasomotor phenomena are importantly related to ocular symptoms in PAPS. Vascular tortuosity was a frequent finding in PAPS patients. Peripheral vascular tortuosity was associated with triple positivity and might be a biomarker of ischemic microvascular retinopathy due to PAPS.

Ophthalmologic manifestations in primary antiphospholipid syndrome patients: A cross-sectional analysis of a primary antiphospholipid syndrome cohort (APS-Rio) and systematic review of the literature.

OBJECTIVE: There is a broad spectrum of eye involvement in antiphospholipid syndrome (APS). The majority of descriptions are presented as case reports that include mostly APS patients secondary to systemic lupus erythematosus (SLE), with no compelling evidence in primary APS (PAPS). This study aimed to describe ocular manifestations in our well-defined PAPS cohort (APS-Rio) and then perform a systematic literature review (SLR) of ocular manifestations in patients with APS or positivity to aPL without SLE. METHODS: We retrospectively analyzed PAPS patients followed at our outpatient clinics. All patients fulfilled Sydney APS classification criteria (2006). We evaluated them for ocular symptoms and previous ocular diagnoses. Antiphospholipid antibodies and clinical APS manifestations were compared between patients with and without ocular manifestations. For the SLR, electronic databases were searched up to November 2019. RESULTS: We studied 105 PAPS patients; 90.5% were female and 56.2% were Caucasian. We found ocular manifestations in 37.1% of our cohort. Thrombosis was the main criteria manifestation (95.2%) and lupus anticoagulant was the most prevalent antibody. Ophthalmologic diagnoses were present in 7 patients, with 5 having retinal vessels thromboses. Amaurosis fugax was the leading complaint, present in 30 patients. In the univariate analysis, amaurosis fugax was related to livedo (p = 0.005), Raynaud's phenomenon (p = 0.048) and the presence of anticardiolipin antibody (≥40 GPL/MPL) (p = 0.041). Hemianopia was associated with arterial hypertension (p = 0.049). In the multivariate analysis, the only association found was between livedo and amaurosis fugax (OR 4.09, 95%CI 1.5-11.11, p = 0.006). Our SLR incorporated 96 articles of ocular manifestations in patients with PAPS or positivity to aPL without SLE. Ocular findings varied from 5 to 88%, including anterior and posterior segments, orbital and neuro-ophthalmologic changes. CONCLUSION: There is little evidence on ocular manifestations in PAPS. We described an association between livedo and amaurosis fugax. Prospective studies are needed to promote the best treatment and avoid blindness in PAPS patients.