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Nonarteritic anterior ischemic optic neuropathy as the presenting manifestation of primary antiphospholipid syndrome


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Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis or pregnancy morbidity. Ocular involvement in APS includes a broad spectrum of manifestations involving anterior and posterior segment or the presence of neuroophthalmologic features. Nonarteritic anterior ischemic optic neuropathy (NAION) is a very rare finding, and in this report a case having NAION as the prevailing sign of APS is presented. A middle-aged women who presented with visual disturbances in her left eye (LE) and turned out to have the diagnosis of primary APS with the help of rheumatological investigations is discussed. She was treated with oral steroids for NAION in her LE. With systemic and rheumatological work-up, primary APS was diagnosed, and hydroxychloroquine, coumadin, and aspirin were started, after which she remained stable under control. Due to the important diagnostic and therapeutic implications of APS, it should be considered in the differential diagnosis of NAION, particularly when the etiology is uncertain.
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Indian J Ophthalmol. May 2014; 62(5): 642–644.
doi: 10.4103/0301-4738.109535
PMCID: PMC4065524
Nonarteritic anterior ischemic optic neuropathy as the presenting
manifestation of primary antiphospholipid syndrome
Betul Tugcu, Nur Acar,1 Cigdem Tanrıverdi Coskun, Selda Celik,2 and Fadime Ulviye Yigit
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This article has been cited by other articles in PMC.
Nonarteritic anterior ischemic optic neuropathy (NAION) has been reported in association
with a number of systemic conditions. However, it has rarely been reported with
antiphospholipid syndrome (APS).[1] APS is an autoimmune disorder characterized by
vascular (arterial, venous, or small vessel) thrombosis or pregnancy morbidity. Diagnosis
of APS is based on having at least one clinical (vascular occlusion and/or pregnancy
complications) finding and one laboratory criterion (repeated positivity for lupus
anticoagulants and/or antibodies targeted against cardiolipin or β (2)-glycoprotein in 3
months) at the same time.[2] Ocular involvement in APS includes a broad spectrum of
manifestations from the anterior and posterior segment, or the presence of
neuroophthalmologic features. Neuroophthalmologic features are transient visual loss,
ischemic optic neuropathy (ON), and progressive optic nerve atrophy.[3]
NAION has rarely been reported as the prevailing sign of APS. We here described a case
presenting with NAION in whom a primary APS was subsequently diagnosed.
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Case Report
A 36 year old Caucasian woman was admitted because of headache and sudden unilateral
visual loss in her left eye (LE). She was not a smoker or alcohol consumer. The patient
did not have hypertension, hyperlipidemia, and diabetes mellitus. She had a history of
abortion in the second trimester. Physical examination was normal.
Ophthalmological examination showed a left relative afferent pupillary defect (RAPD)
and swelling of the left optic disc (OD) with accompanying pallor. Visual acuities (VA)
were 20/20 and 6/20 in the right and LE, respectively. Color vision was impaired in the
LE with Ishihara color plates. Visual field testing with automated perimetry (Humphrey
Field Analyser Model 750i, HFA II, Carl Zeiss Meditec Inc. Dublin, CA, USA; 1994-
2000 central 30-2 threshold test) showed a dense inferior arcuate defect [Fig. 1].
Examination of the right eye was normal. Initially, a diagnosis of ON was made and
intravenous methylprednisolone was started and given 1 g/day for 3 consecutive days.
Oral prednisolone 1 mg/ kg/ day was started on day 4 and continued for 1 week and
stopped in 1 month by tapering the dose each week. VA increased to 0.7 on day 1, to 0.9
on day 2, and to 1.0 at week 1.
Figure 1
Visual field test demonstrates an inferior arcuate defect in the LE
At follow-up examination at the end of the first month, the visual acuity in the LE was
20/20 and pupillary light reflexes were normal. Cup/disc (c/d) ratio was analyzed with
Optical Coherens Tomography (Optovue Fourier domain OCT, Optovue Inc, Freemont,
CA, USA). Horizontal c/d ratio was 0.54 in RE and 0.40 in LE. Vertical c/d ratio was 0.32
in RE, and 0.41 in LE. OD edema in LE resolved leaving partial atrophy in the superior
part of the optic disc and the visual field test revealed partial improvement of the inferior
arcuate defect. Corticosteroid therapy was stopped. Two months later, she presented again
with blurred vision in her LE. The VAs were 20/20 in both eyes, with no RAPD. The OD
had a slight pallor superiorly [Fig. 2]. Fluorescein angiography showed a patchy
background choroidal flourescence and a relative delay in arterio-venous filling in the
superior temporal retinal vessels compared with the inferior temporal ones [Fig. 3]. No
leakage from the disc or vessels was observed.
Figure 2
Fundus photograph of the LE. Note the optic disk pallor in the superior half
Figure 3
Fundus fluorescein angiography of the LE. Fluorescein angiography showed a patchy
background choroidal flourescence and a relative delay in arterio-venous filling in the
superior temporal retinal vessels compared with the inferior temporal ones in the ...
Extensive investigations for hypertension, hyperlipidemia, diabetes mellitus,
atherosclerosis, cardiac diseases connective tissue, and autoimmune disorders were
performed. As there was no clinical signs or findings of infectious disease, no extensive
work-up was performed. Renal and liver tests, glucose, and lipid profile were all normal.
Factor V Leiden and prothrombin gene mutations were absent. SLE and other connective
tissue disorders were excluded with intensive investigations. APS was detected in the
immunological examination [lupus anticoagulant 62.9 s (normal 31-44); anticardiolipin
antibodies IgG, 18.86 (normal 0-10), IgM 30.90 (normal 0-7)]. Primary APS was
diagnosed based on the above clinical and laboratory findings and treatment with oral
anticoagulant (coumadin 5 mg/day), antiagregan (coraspin 100 mg/day), and
antiinflammatory drug (hydroxychloroquine 200 mg/day) was started immediately. We
tested lupus anticoagulant and anticardiolipin antibodies IgG and IgM 3 months later, and
they were still at high levels, which confirmed the diagnosis.
The patient remained asymptomatic under treatment. One year later, the patient had 20/20
vision in both eyes and did not have any new thrombotic events. She is still under the
above-mentioned treatment with excellent treatment efficacy regarding the INR values
and without side effects.
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The systemic features of the APS syndrome are characterized by large variability
depending on the affected organs. However, studies on the frequency and clinical
presentation of the ocular manifestation as the presenting sign of APS in patients suffering
from unexplained ocular disease are missing.[4] Herein, we present a case of NAION as
the first manifestation of APS, which is very rarely described.
Ocular involvement associated with APS include vaso-occlusive retinopathy and ON.[5]
Prevalence of ocular vaso-occlusive disease has been estimated in 29% of patients with
primary APS, but ON is a very rare manifestation of primary APS and only a few isolated
cases have been reported in the literature.[6] Tsironi et al.[4] reported a case series with
ocular disorders as the prevailing manifestations of APS. The first patient had bilateral
retinal occlusive disease and the second and third patient had unilateral NAION with
macular edema.[4] Our case was also unilateral, which responsed to CS therapy with
recurrence of blurred vision after its cessation. Reino et al.[1] reviewed cases of ON in
APS from the recent literature. ON was associated with systemic thrombotic
manifestations, most commonly with neurological disorders followed by cardiac disease,
arterial and venous thrombosis, miscarriage, and livedo reticularis.[1] However, in our
case there was no systemic thrombotic events. The only important finding was her history
of abortion. The most probable pathogenesis of ON is an abnormal microcirculation
secondary to thrombosis of the small arterial vessels that arise from the choroidal
circulation and supply the anterior portion of optic nerve caused by antiphospholipid
antibodies, similar to other clinical manifestations of the primary APS.[7] Giorgi and
Gabrieli[2] suggested that a monolateral ON in patients with significant levels of aPL
could be considered as a focal lesion due to microthrombotic events involving the ciliary
Treatment of ON in primary APS is controversial and should be individually tailored.
Anticoagulation therapy can prevent future events not only in eyes but also episodes in
other vital organs.[5,8] As thrombosis with no evidence of vasculitis seems to be probably
the most important mechanism of this ON, treatment with aspirin or anticoagulants must
be tried, although in several occasions prednisolone has also been used. Corticosteroids
have no established role in the treatment of APS but are used for rheumatic symptoms in
patients with accompanying systemic autoimmune illness. However, high doses of
corticosteroids are usually given empirically to patients with severe thrombocytopenia,
hemolytic anemia, and catastrophic APS.
Our patient received systemic steroids because of the initial diagnosis of ON. As APS was
diagnosed during rheumatologic work-up 2 months after the completion of the steroid
therapy, anticoagulant therapy with antiinflammatory drug hydroxychloroquine was
started. The protective effect of hydroxychloroquine on thrombosis in systemic lupus
erythematosus patients has been shown in the literature. Furthermore, use of this drug has
been considered as a new approach on prevention of the thrombosis in the APS.[9,10]
In conclusion, albeit rarely ON may be observed as a presenting manifestation in primary
APS, it is advisable that patients with ON in whom the etiology is uncertain may be tested
for the presence of antiphospholipid antibodies. Due to the important diagnostic and
therapeutic implications of APS, the possibility of ocular features as the first clinical
manifestation of APS, particularly in patients with no evident risk factors for ocular
disease, should be kept in mind of ophthalmologists.
... However there are few reports in which it appears as NAION. In APS cases reported by Serrador-García et al. [4] and Tugcu et al. [5] , NAION occurred unilaterally. However, no study reported bilateral NAION caused by APS. ...
... Standard treatment strategy of APS is reducing thrombotic risk [1] . There are reports about use of warfarin, antiplatelets, anti-inflammatory drug and hydroxychloroquine, after APS diagnosis [5] . Authors also started using warfarin after confirming blood abnormality, and intensively used antithrombotic agents intravenously at the second episode, but the prognosis was poor. ...
... Выраженность клинических и серологических маркеров первичного АФС может быть весьма разнообразной -от угрожающих жизни состояний до минимальных расстройств. Глазные проявления первичного и вторичного вариантов антифосфолипидного синдрома, обнаруживаясь примерно в 37 % случаев, разнообразны и включают в себя вазоокклюзивную нейроретинопатию, некротизирующий склерит, кератит и увеит [16][17][18][19][20]. ...
Introduction. Antiphospholipid syndrome (APS) is one of the leading causes of nonarteritic anterior ischemic opticoneuropathy (AION) in young patients. Ocular pathology, often being the earliest and the only manifestation of APS, explains possible difficulties in diagnosis of this thrombophilic condition. Objective – to present a clinical case of bilateral anterior ischemic opticoneuropathy against the background of newly diagnosed antiphospholipid syndrome in a young patient. Materials and Methods. At admission, the patient, born in 1988, was diagnosed with bilateral acute ischemic nonarteritic ION on the basis of visual acuity decrease to 0,7, narrowing of peripheral borders of visual fields concentrically by 10–15°, optic disc edema (ODE) of both eyes and macular edema of the right eye according to ophthalmoscopy and optical coherence tomography (OCT). Laboratory examination revealed thrombocytopenia, antibodies to DNA and cardiolipin in elevated titer, which allowed the diagnosis of primary antiphospholipid syndrome. Local anticoagulant and anticoagulant therapy, systemic administration of antioxidants, antiaggregants, vitamins, as well as preparations, improving microcirculation, were carried out. Results and Discussion. Against the background of treatment visual function restored to 1.0 in the right eye and 0.9 in the left eye, residual ODE edema was diagnosed in both eyes in the upper nasal sector. At follow-up in 2.5 months the morphological parameters of the retina and optic nerve were restored. Conclusion. Thromboocclusive lesions of retinal and optic nerve vessels are an early and sometimes the only manifestation of antiphospholipid syndrome. Their presence in young patients requires clinical and laboratory follow-up examination for timely diagnosis of concomitant thrombophilic condition. High risk of recurrent thrombosis of different localization makes it necessary to take antiplatelet agents for a long time.
... 16 Nonarteritic anterior ischemic optic neuropathy, retrobulbar neuritis, and frosted branch angitis are also reported in primary APS. [18][19][20] Human retina receives blood supply by two main circulations, retinal and choroidal blood vessels. 21 Systemic diseases affecting the vascular system can affect the eye by causing damage to retinal and/or choroidal blood supply. ...
Background & Objectives: Antiphospholipid syndrome can lead to variable systemic and ophthalmic manifestations. The study aims to establish the relationship between retinal vein occlusion (RVO) or choroidal neovascular membrane (CNVM) with Antiphospholipid Syndrome (APS). Methods: A multicentric prospective cross-sectional study was carried out in three major eye centers in Bahrain that included The Eye Infirmary, Bahrain Royal Hospital, and King Hamad University Hospital. All young patients aging from 35 to 50 years, presenting with either RVO or CNVM and not known to have any underlying systemic disease were included in the study. Antiphospholipid confirmatory tests and coagulation profiles were done for all patients. Results: Total number of fourteen eyes of ten patients were enrolled in the study. Six patients had RVO, three out of them had central retinal vein occlusion (CRVO) and three had branch retinal vein occlusion (BRVO). Four patients had bilateral CNVM (total of 8 eyes). All patients recorded high activated partial thromboplastin time (aPTT) readings, six out of ten patients were labeled as primary APS, four of them had positive anti-cardiolipin antibodies and the other two patients were positive for plasma lupus anticoagulant. Conclusion: APS should be considered as an etiological factor in all cases of retinal vein occlusion and choroidal neovascular membrane affecting young otherwise healthy individuals. In this study, 60% of patients who fit the inclusion criteria were labeled as primary APS patients. Timely investigations and treatment in these cases can prevent major catastrophic thrombotic events which may involve any system or organ in the body. Keywords: Antiphospholipid syndrome, Blood coagulation tests, Eye, Lupus coagulation inhibitor, Retinal vein occlusion
... pacjentów młodych, bez dodatkowych czynników ryzyka(1,13). Katastrofalny APS stanowi rzadką (około 1% chorych z APS), ale często śmiertelną postać APS, przebiegającą z ostrą niewydolnością wielonarządową (najczęściej ≥3 narządów) z powodu ostrej mikroangiopatii zakrzepowej, pojawiającą się w krótkim przedziale czasu. Chociaż do zajęcia tętnicy środkowej siatkówki dochodzi stosunkowo rzadko (ok. ...
Background Antiphospholipid syndrome is an autoimmune disorder characterized by recurrent vascular thrombosis and pregnancy losses in the presence of persistently positive antiphospholipid antibodies. Bibliometric analysis and altmetric analysis are methods of analyzing academic productivity and influence. Currently, the assessment of antiphospholipid syndrome through the above analyses is lacking. This study investigated the quantity and quality of studies in the field of antiphospholipid syndrome and revealed the characteristics of worldwide productivity on this disease by the bibliometric analysis and altmetric analysis.Methods The terms “APS,” “antiphospholipid syndrome,” “antiphospholipid-antibody syndrome,” and “Hughes syndrome” were searched on the Web of Science Core Collection from January 1, 2011, to December 31, 2021. Original articles and reviews were selected. We set the filters as “English.”Results and conclusionA total of 1818 articles were retrieved from 68 countries, of which 20 met the criteria of major active countries. High-income countries contributed 1341 articles (73.48%). The number of articles annually increased significantly in the 10-year period (P < 0.001). The USA (253, 13.91%) was the most productive country. Adjusted by population, Serbia was top of the list. According to the gross domestic product analysis, Serbia ranked first. The most used keywords such as thrombosis and antiphospholipid antibodies were presented by keywords analyses. A content analysis found antithrombotic and anticoagulant therapy as research hotspots. A significant correlation was detected between average citations and altmetric attention scores (P = 0.002) and Mendeley readers count (P < 0.001). From 2011 to 2021, the number of global articles increased rapidly. Most papers came from high-income countries. The relationship between the bibliometric and altmetric analyses were basically consistent; therefore the two can prove/complement each other. Key points • We revealed the global productivity characteristics of the papers related to antiphospholipid syndrome by using the methods of bibliometric analysis and altmetric analysis. • We found the most selected articles that describe the treatment of antiphospholipid syndrome, especially antithrombotic and anticoagulant treatments, which may be the current research hotspot.
Introduction Non-arteritic anterior ischemic optic neuropathy (NAION) is the most common cause of optic nerve swelling and optic neuropathy in adults over 50 years of age. It has been rarely reported during pregnancy, mostly related to systemic and ocular predisposing conditions. Case Report We report the case of a 44 years-old healthy female with no previous remarkable clinical history. She had in vitro fertilisation treatment to get pregnant. 18 days after uneventful cesarean section she referred sudden painless vision loss in her right eye (RE), denser inferiorly, with concurrent optic disc edema and relative afferent pupillary defect. Steroid intravenous treatment during the acute episode showed no improvement. Our patient showed normal magnetic resonance imaging (MRI), blood test, autoimmune disease biomarkers, infectious serology and inflammatory markers. She was diagnosed of RE NAION. After one year follow-up visual field defect remains stable. Conclusion As far as we know this is the first report of NAION after in vitro fertilisation, uneventful pregnancy and cesarean section showing no systemic or ocular risk factors other than a small cup to disc ratio. Hemodynamic and hormonal changes during late pregnancy and uneventful cesarean section can trigger an episode of NAION in a healthy young woman.
Background: Antiphospholipid syndrome (APS) is characterized by several clinical manifestations such as venous and arterial thrombosis associated with persistent antiphospholipid antibodies (aPL). Several studies confirmed that retinal vein occlusion was the most common APS ocular manifestation. The purpose of this study was to identify ophthalmologic manifestations in a homogeneous cohort of well-defined persistently aPL-positive patients and to determine variables associated with these manifestations. Methods: APL-positive patients were selected from two research programs. All ophthalmologic manifestations including those related to APS were recorded. Results: A total of 117 patients were included and 10 of them had APS-related ophthalmologic manifestations (glaucoma, hydroxychloroquine-related maculopathy, anterior acute uveitis, anterior ischemic optic neuropathy). Systemic Lupus Erythematosus (SLE) (OR = 3.4[95%CI; 0.9-12.7), corticosteroids (OR = 9.0 [95%CI; 2.2-37.7]) and aPL-related nephropathy (OR = 7.1 [95%CI; 1.7-30.0]) were significatively associated with the risk of APS-related ophthalmologic manifestations. Conclusion: Most of ocular manifestations in this study were iatrogenic related to corticosteroids or hydroxychloroquine. Patients with SLE, small vessel thrombosis in general, or with aPL-related nephropathy in particular, seemed at higher risk to develop APS-related ophthalmologic manifestations thus deserving adequate monitoring.
Systemic lupus erythematosus can affect any organ system at presentation and throughout its course. Lupus retinal vasculitis is responsible for visual loss of variable severity and typically ascribed to the involvement of pre-capillary superficial arterial vasculature. Using multimodal imaging techniques and electrophysiology, we demonstrate the first ever case of severe lupus retinal vasculitis resulting in the rare association of paracentral acute middle maculopathy. The findings showcase the involvement of both superficial and deep retinal capillary vasculature, and the localization of retinal dysfunction. This precise evaluation facilitated the timely use of combination immunomodulatory therapy with localized pan-retinal laser photocoagulation, resulting in preservation of vision.
The neuropsychiatric involvement in systemic lupus erythematosus (NPSLE) is a challenge for clinicians, both at a diagnostic and therapeutic level. Although in in 1999 the American College of Rheumatology (ACR) proposed a set of definitions for 19 NPSLE syndromes, with the intention of homogenizing the terminology for research purposes and clinical practice, the prevalence of NPSLE varies widely according to different series and is estimated to be between 37 and 95%. This is due to multiple factors such as the unalike definitions used, the diverse design of the studies, type of population, race, type and severity of symptoms, and follow-up of the different cohorts of patients with SLE. In recent years, some authors have tried excluding minor neuropsychiatric manifestations in order to try to reduce this wide variation in the prevalence of NPSLE since they are very prevalent in the general population; others authors have developed various models for the attribution of neuropsychiatric events to SLE that can assist clinicians in this diagnostic process, and finally, some authors developed and validated in 2014 a new algorithm based on the definitions of the ACR that includes the evaluation of the patient's lupus activity together with imaging techniques and the analysis of cerebrospinal fluid (CSF), with the aim of trying to differentiate the true neuropsychiatric manifestations attributable to SLE. In 2010, the European League Against Rheumatism (EULAR) developed recommendations for the management of NPSLE. We found abundant literature published later where, in addition to the recommendations for the management of the 19 NPSLE syndromes defined by the ACR, additional recommendations are given for other neurological and/or psychiatric syndromes, conditions, and complications that have been associated to SLE in recent years. We review below the diagnostic and therapeutic management of the different entities.
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Antiphospholipid syndrome is an autoimmune disorder characterized by either a history of vascular thrombosis (one or more clinical episodes of arterial, venous, or small vessel thrombosis in any tissue or organ) or pregnancy morbidity in association with the presence of antiphospholipid antibodies. The systemic features of the syndrome are characterized by large variability depending on the affected organ(s). Among them, neurological and behavioural disturbances, dermatological features as livedo reticularis and renal, ocular, liver or valvular heart manifestations have been reported in antiphospholipid syndrome patients. However, studies on the frequency and clinical presentation of the ocular manifestations as the prevailing (first) sign of antiphospholipid syndrome in patients suffering from "unexplained" ocular disease are missing. Herein, we present three cases suffering from unexplained ocular disease as first manifestation of antiphospholipid syndrome. All the three patients were referred to our department because of unexplained ocular features from the anterior or posterior segment and unexplained neuro-ophthalmologic symptoms. The first patient had bilateral retinal occlusive disease, the second and the third patient had unilateral nonarteritic anterior ischemic optic neuropathy with macular oedema. Moderate to high levels of antiphospholipid antibodies were detected in all of them at baseline as well as 6 to 12 weeks after initial testing confirming the presence of antiphospholipid antibodies. Anticoagulant treatment with acenocoumarol was instituted resulting in stabilization and/or improvement of ocular signs in all of them. Due to the important diagnostic and therapeutic implications of antiphospholipid syndrome, the possibility of ocular features as the first clinical manifestation of antiphospholipid syndrome should be kept in mind of the physicians particularly in patients with no evident risk factors for ocular disease. In this case, prompt anticoagulant treatment and close follow-up seem to be essential for vision salvation and stabilization.
Studies have shown a protective effect of hydroxychloroquine on thrombosis in systemic lupus erythematosus patients. Recent in vitro studies have demonstrated that this molecule was able to restore the anticoagulant action of annexin A5, which is reduced in presence of antiphospholipid antibodies. Hydroxychloroquine use may be a new approach of the prevention of thrombosis in the antiphospholipid syndrome, which remains to be validated by well-conducted clinical trials. Copyright © 2010 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.
Persistently positive antiphospholipid antibodies in association with thromboses and/or pregnancy morbidity is the hallmark of the antiphospholipid syndrome. The management of antiphospholipid antibody-positive patients has been focused on utilizing anti-thrombotic medications such as heparin or warfarin. Given that our understanding of the molecular mechanisms of antiphospholipid antibody-mediated thrombosis has been growing, it is highly likely that the current 'anti-thrombotic' approach to these patients will be replaced by an 'immunomodulatory' approach in the near future. This review article will address the experimental and/or clinical evidence behind some of these potential 'immunomodulatory' approaches (tissue factor inhibition, P38 mitogen-activated protein kinase inhibition, nuclear factor-kappaB inhibition, platelet glycoprotein receptor inhibition, hydroxychloroquine, statins, inhibition of beta(2)GPI and/or anti-beta(2)GPI binding to target cells, complement inhibition, and B cell inhibition) in antiphospholipid syndrome.
Four patients with isolated acute ocular ischemic syndromes also had circulating antiphospholipid antibodies. Two patients had vaso-occlusive retinopathy and two, anterior ischemic optic neuropathy (which was successive in one). Extensive clinical laboratory evaluation identified vascular risk factors in two patients. One patient had essential thrombocytosis, confirmed by bone marrow biopsy; the other had stable hypertension and a history of coronary artery disease. These cases suggest that small vessel thrombosis in situ may be a mechanism for antiphospholipid-associated ocular and cerebral ischemia.
The Hemostasis and Thrombosis Laboratory at the Oregon Health Sciences University identified 80 patients with significantly elevated anticardiolipin antibody (ACLA) levels. We reviewed all of their available medical records and found that 25 of these patients had associated neurological symptoms or disorders. These symptoms and disorders could be grouped into four distinct clinical patterns comprising encephalopathy, multiple cerebral infarctions, migraine-like headaches, and visual abnormalities including amaurosis fugax and ischemic optic neuropathy. Cerebral ischemia best explained these neurological dysfunctions. There was no correlation between the presence or absence of neurological disease and ACLA levels, but ACLA levels were higher in patients with encephalopathy than in others with neurological involvement (p less than 0.05). How neurological dysfunction and the presence of these antiphospholipid antibodies are related remains to be clarified. Nevertheless, in patients with unexplained cerebral ischemia, establishing the presence of ACLA may have prognostic and therapeutic importance. In particular, acute immunosuppressive therapy and plasmapheresis may be useful in patients with acute ischemic encephalopathy.
The lupus anticoagulant is an acquired serum immunoglobulin that prolongs several coagulation parameters, most notably the partial thromboplastin time (PTT). Most commonly, this condition is found in association with systemic lupus erythematosus (SLE) but may be seen with other collagen-vascular diseases and in otherwise healthy individuals. Despite the laboratory tests suggesting impaired coagulation, clinically the lupus anticoagulant has been associated with thrombosis. The authors present five patients with the lupus anticoagulant who came to medical attention because of branch retinal artery occlusion, ischemic optic neuropathy, transient visual loss, transient diplopia, or vertebrobasilar insufficiency. Eleven previously reported patients with the lupus anticoagulant and disturbed vision are also reviewed with additional findings of retinal venous occlusive disease and homonymous visual field loss. The relationship of these findings to retinopathy in SLE is discussed. Patients with the lupus anticoagulant (with or without SLE) may develop disturbances in vision due to thrombosis from a hypercoagulable state. We recommended obtaining a PTT, VDRL, and the more sensitive anticardiolipin antibody in patients with unexplained visual symptoms.
Since the original description and definition of the antiphospholipid syndrome (APS), a number of distinct clinical manifestations related to it have appeared in the literature. These include vascular obstruction of both veins and arteries, thrombus formation on the endocardium and its consequences, as well as a group of other conditions where vascular obstructive mechanisms are either incompletely understood or unproven, eg, chorea, avascular necrosis, and pulmonary hypertension. Single vessel (large/medium) involvement or multiple vascular occlusions may cause a wide variety of presentations. Any combination of vascular occlusive events may occur in the same individual, and the time interval between them also varies considerably from weeks to months or even years. Rapid chronological occlusive events occurring over days to weeks have been termed the "catastrophic" APS. Most of these complications may be ascribed to the hypercoagulable state of which antiphospholipid antibodies appear either to be "markers" or intimately connected with the highly complex coagulation mechanisms resulting in thrombotic occlusions.
Several visual disturbances have been associated with the presence of antiphospholipid antibodies, including vaso-occlusive retinopathy and ischaemic optic neuropathy. The latter has been rarely described in patients with "primary" antiphospholipid syndrome, and, when present, is generally associated with neurological disorders. We describe the case of a patient presenting with optic neuropathy in whom a "primary" antiphospholipid syndrome was diagnosed, and review all previously reported similar cases. The pathogenesis and treatment are also discussed.
Optic neuropathy is a well-known ocular manifestation occurring in patients with systemic lupus erythematosus (SLE), and it remains one of the major causes of blindness in these patients. We report data from six SLE patients with optic neuropathy, one of whom was considered to have antiphospholipid syndrome (APS). This patient had monolateral optic neuropathy, whereas the other five SLE patients had bilateral optic nerve disease. We believe that the monolateral occurrence of optic neuropathy in our patient can be considered as a 'focal' neurological disease due to a thrombotic event involving the ciliary vasculature. Conversely, bilateral optic nerve damage in SLE could be considered to be a 'general' neurological disease due to different immunological mechanisms, such as vasculitis. Additionally, the literature on SLE patients affected by optic neuropathy is reviewed to evaluate the major clinical features, particularly neurological features. In reviewing the literature, it appears that bilateral optic neuropathy in SLE occurs more frequently than monolateral optic neuropathy, and the main neurological manifestation seen in these patients is transverse myelitis, particularly in SLE patients with bilateral optic nerve disease. Finally, we propose a clinico-ophthalmological spectrum of APS and outline the ocular clinical manifestations that can be considered as diagnostic for the syndrome.
To evaluate ocular features in patients presenting with inflammation in the presence of anticardiolipin antibodies. A descriptive study of 13 patients presenting with idiopathic ocular inflammation involving anterior and posterior segment was performed. Patients were followed for a mean follow-up of 22 months (range, 1 to 125). A comprehensive report of ocular involvement, including visual symptoms, visual acuity, clinical characteristics, funduscopic and fluorangiographic features, was reported. Systemic associated symptoms were analyzed. Laboratory investigations included anticardiolipin antibody titers and isotypes, presence of other autoantibodies, and markers of immune system activation. The most common ocular symptom at presentation was blurred vision (eight patients) followed by redness and pain(three patients) and visual loss(two patients). Anterior segment abnormalities, including iritis (eight patients) scleritis (two patients) and filamentary keratitis (one patient), were present in 76% of patients, whereas the most represented feature of posterior involvement was retinal vasculitis (60%) followed by vitritis (38%), retinal detachment (15%), posterior scleritis (7%), and central retinal artery occlusion (7%). All patients had abnormal titers of anticardiolipin antibodies, predominantly IgG isotype; six had markers of immune system activation. Although posterior pole disease is more commonly associated with anticardiolipin antibodies, the anterior segment can also be involved with a wide spectrum of features. Scleritis has never been previously described as associated with anticardiolipin antibodies. Systemic symptoms are frequently present in association with ocular disease.