Abstract:The use of different types of ERG enable detection of not only local, but also generalized retinal changes in APS. Regardless of the presence of retinal occlusion, APS patients show suppression of biopotential in most of cellular elements of the retina due to ischemia. Despite high visual acuity, the local macular potential gets significantly reduced in more than half of APS patients. Oscillatory potentials recording is a highly sensitive method for diagnosis of retinal ischmia in APS.
“…Finally, electrophysiological activity of the retina was studied in APS patients, showing an abnormal electroretinography with consistently lower a- and b-wave amplitudes in 70% of cases and a decrease in the oscillatory potential in 82% of the patients, probably because of the retinal ischemia. 107…”
Section: Part II – Systematic Review Of Ophthalmological Manifestatiomentioning
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.
“…Finally, electrophysiological activity of the retina was studied in APS patients, showing an abnormal electroretinography with consistently lower a- and b-wave amplitudes in 70% of cases and a decrease in the oscillatory potential in 82% of the patients, probably because of the retinal ischemia. 107…”
Section: Part II – Systematic Review Of Ophthalmological Manifestatiomentioning
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.
Introduction Antiphospholipids syndrome is a rare autoimmune whose Clinical manifestations are very wide and different. Vaso-occlusive manifestations are the most frequent ones. Vessels of all organs can be involved. Ocular involvement is among the rarest. We report a case of retinal arterial thrombosis in the context of APS. Case report A 46-year-old patient with no previous medical history was admitted to the ophthalmology department for visual field amputation of the right eye without other associated signs. On the fundus, the left side was without abnormality and the right side was the site of a mixed arterial and superior temporal venous occlusion. Retinal angiography revealed a retinal focus in the superior temporal region with mixed occlusion of arterial and venous branches with ischemia of the superior temporal quadrant on the right and a retinal focus in the superficial inferior temporal region without occlusion on the left. Optical coherence tomography revealed ischemia of the superficial layers with alteration of the macular microvascularization in the right superior temporal quadrant. Immunological work-up showed antinuclear antibodies to be positive at 160 and anti-bgp1 positive with an IgG level of 118 IgM higher than 118 IgA at 101. The patient was put on effective anticoagulation with good clinical evolution. Conclusion Ocular involvement is rare in APS and associated with poor prognosis, it seems to be difficult to diagnose but also to manage. Larger studies are required to establish guidelines on how and when to screen asymptomatic APS patients for ocular damage, but also on how to prevent and treat it.
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