Ophthalmologic manifestations in patients with antiphospholipid antibodies: Beware of iatrogenic complications

Lupus ◽  
2021 ◽  
pp. 096120332110339
Author(s):  
Justine Menet ◽  
Nelly Agrinier ◽  
Virginie Dufrost ◽  
Jean-Baptiste Conart ◽  
Denis Wahl ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Clinical Manifestations ◽  
Ischemic Optic Neuropathy ◽  
Ocular Manifestations ◽  
Uveitis Anterior ◽  
Vein Occlusion ◽  
Iatrogenic Complications ◽  
Vessel Thrombosis

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.

scholarly journals Case Report: Recurrent retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 761
Author(s):  
Marwa Ben Brahim ◽  
Sondes Arfa ◽  
Fadia Boubaker ◽  
Jihen Chelly ◽  
Wafa Ammari ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Retinal Vein Occlusion ◽  
Male Patient ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Ocular Manifestations ◽  
Vein Occlusion ◽  
First Case

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the revealing manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

scholarly journals Case Report: Retinal vein occlusion as the first clinical manifestation of systemic lupus erythematosus in a male patient

F1000Research ◽  
2021 ◽  
Vol 10 ◽  
pp. 761
Author(s):  
Marwa Ben Brahim ◽  
Sondes Arfa ◽  
Fadia Boubaker ◽  
Jihen Chelly ◽  
Wafa Ammari ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Retinal Vein Occlusion ◽  
Male Patient ◽  
Lupus Erythematosus ◽  
Clinical Manifestations ◽  
Systemic Lupus ◽  
Initial Manifestation ◽  
Ocular Manifestations ◽  
Vein Occlusion ◽  
First Case

Systemic lupus erythematosus (SLE) is a chronic, autoimmune disease characterized by widespread clinical manifestations and immunological disorders. A myriad of ocular manifestations can be seen in patients with SLE. The most vision-threatening complication is vaso-occlusive retinopathy including retinal vein occlusion (RVO). RVO associated with SLE is well described in the literature and its association with antiphospholipid antibodies is recognized. However, RVO as the initial manifestation of SLE is scarcely reported. Herein, we report the first case of recurrent RVO as the primary manifestation of SLE in a 40-year-old male patient. He had two consecutive episodes of decreased vision. Ophthalmologic examination disclosed a branch retinal vein occlusion the first time and a central retinal vein occlusion the second time. The diagnosis of SLE was established based on clinical and immunological criteria. He was prescribed antiplatelet therapy, hydroxychloroquine at 5.5 mg/kg/day, and intravitreal anti-vascular endothelial growth factor (VEGF) antibodies regimen. He slowly improved under treatment.

scholarly journals Current concepts in the diagnosis and management of antiphospholipid syndrome and ocular manifestations

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Gunay Uludag ◽  
Neil Onghanseng ◽  
Anh N. T. Tran ◽  
Muhammad Hassan ◽  
Muhammad Sohail Halim ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Autoimmune Disorder ◽  
Ocular Involvement ◽  
Ocular Manifestations ◽  
Different Types ◽  
Thrombotic Complications ◽  
Specific Factors ◽  
Arteries And Veins

AbstractAntiphospholipid syndrome (APS) is an autoimmune disorder associated with obstetrical complications, thrombotic complications involving both arteries and veins, and non-thrombotic manifestations affecting multiple other systems presenting in various clinical forms. Diagnosis requires the presence of antiphospholipid antibodies. The exact pathogenesis of APS is not fully known. However, it has recently been shown that activation of different types of cells by antiphospholipid antibodies plays an important role in thrombosis formation. Ocular involvement is one of the important clinical manifestations of APS and can vary in presentations. Therefore, as an ophthalmologist, it is crucial to be familiar with the ocular findings of APS to prevent further complications that can develop. Furthermore, the ongoing identification of new and specific factors contributing to the pathogenesis of APS may provide new therapeutic options in the management of the disease in the future.

Childhood-Onset Systemic Lupus Erythematosus: Antiphospholipid Antibodies in 37 Patients and Their First-Degree Relatives

PEDIATRICS ◽  
1993 ◽  
Vol 92 (6) ◽  
pp. 849-853
Author(s):  
Charles Molta ◽  
Olivier Meyer ◽  
Christine Dosquet ◽  
Marcela Montes de Oca ◽  
Marie-Claude Babron ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Fetal Loss ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
First Degree Relatives ◽  
Antiphosphatidylethanolamine Antibodies ◽  
Onset Systemic Lupus Erythematosus

Objective. Antiphospholipid antibodies (aPL) are noted with increased frequency in patients with systemic lupus erythematosus (SLE). The main manifestations found to be associated with aPL are arterial and venous thrombotic events, thrombocytopenia, and recurrent pregnancy loss This study is an attempt to define the incidence of aPL in patients with childhood-onset SLE and in their relatives and to correlate their presence with clinical manifestations, and especially, to evaluate the risk of thrombosis in aPL-positive subjects. Methodology. We studied 37 unrelated patients and 107 of their first-degree relatives. VDRL, IgG and IgM anticardiolipin, and IgG antiphosphatidylethanolamine antibodies were studied in all probands during periods of clinical remission and in first-degree relatives at the time of interview. Lupus anticoagulant had only been studied in probands during an SLE flare-up. Results. Thirty-eight percent of probands and 19% of relatives were positive for at least one aPL, with little over-lap between the different aPL studied. -No aPL-negative proband developed thrombosis. Two of the aPL-positive probands had thrombotic events before testing, and a third one showed thrombosis after testing. Only two probands had high levels of IgG aCL and showed thrombosis. The occurrence of aPL positivity in relatives was not always related to its presence in probands. None of the aPL-positive relatives had hadthrombosis, but recurrent fetal loss was noted in one aPL-positive mother with SLE. Although there was a high frequency of SLE, SLE-like disease, auto-immune disorders or positive serological findings for lupus in first-degree relatives, many of these relativew did not test positive for aPL. Conclusion. The high levels of IgG aCL may be considered a risk factor for thrombosis. Findings in relatives suggest a multifactorial origin for autoimmune disease and antibody production.

Determinants of Risk for Venous and Arterial Thrombosis in Primary Antiphospholipid Syndrome and in Antiphospholipid Syndrome with Systemic Lupus Erythematosus

2009 ◽  
Vol 36 (6) ◽  
pp. 1195-1199 ◽  
Author(s):  
ADRIANA DANOWSKI ◽  
MARIO NEWTON LEITÃO de AZEVEDO ◽  
JOSE ANGELO de SOUZA PAPI ◽  
MICHELLE PETRI
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Venous Thrombosis ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Pregnancy Loss ◽  
Fold Increase ◽  
Systemic Lupus ◽  
Hereditary Thrombophilia

Objective.Antiphospholipid syndrome (APS) is characterized by thrombosis (venous and arterial) and pregnancy loss in conjunction with the lupus anticoagulant, IgG or IgM anticardiolipin, or IgG or IgM anti-ß2-glycoprotein I. In most series, only a minority of patients with antiphospholipid antibodies develop a clinical manifestation.Methods.A cross-sectional study of consecutive patients in the Hopkins Lupus Center was performed. Interviews were done and records were reviewed for the following variables: gender, ethnicity, hypertension, triglycerides, cholesterol, smoking, diabetes mellitus, homocysteine, cancer, hepatitis C, hormone replacement therapy/oral contraceptives, hereditary thrombophilia, anticardiolipin antibodies IgG, IgM and IgA, and lupus anticoagulant (LAC). Our aim was to identify risk factors associated with thrombosis and pregnancy loss in patients with antiphospholipid antibodies.Results.A total of 122 patients (84% female, 74% Caucasian) were studied. Patients were divided into 3 groups: primary APS, APS associated with systemic lupus erythematosus, and patients with systemic lupus erythematosus (SLE) with antiphospholipid antibodies but no thrombosis or pregnancy loss. Venous thrombosis was associated with high triglycerides (p = 0.001), hereditary thrombophilia (p = 0.02), anticardiolipin antibodies IgG > 40 (p = 0.04), and LAC (p = 0.012). Hypertriglyceridemia was associated with a 6.4-fold increase, hereditary thrombophilia with a 7.3-fold increase, and anticardiolipin IgG > 40 GPL with a 2.8-fold increase in the risk of venous thrombosis. Arterial thrombosis was associated with hypertension (p = 0.008) and elevated homocysteine (p = 0.044). Hypertension was associated with a 2.4-fold increase in the risk of arterial thrombosis. No correlations were found for pregnancy loss.Conclusion.The frequency of thrombosis and pregnancy loss is greater in APS associated with SLE than in primary APS. Risk factors differ for venous and arterial thrombosis in APS. Treatment of hypertension may be the most important intervention to reduce arterial thrombosis. Elevated triglycerides are a major associate of venous thrombosis, but the benefit of treatment is not known. Hereditary thrombophilia is an associate of venous but not arterial thrombosis, making it cost-effective to investigate only in venous thrombosis.

No Thrombotic Risk in Patients with Asymptomatic Antiphospholipid Antibodies

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5254-5254
Author(s):  
Krystyna M Zawilska ◽  
Agata M Kopydlowska
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Study Group ◽  
Hemorrhagic Diathesis ◽  
Thromboembolic Events ◽  
Systemic Lupus ◽  
Thrombotic Risk ◽  
Abnormal Results

Abstract Abstract 5254 Aim of the study. Antiphospholipid antibodies (APA) are well known risk factor for thromboembolic events and/or obstetric complications. They may be found incidentally in patients without thrombotic complications (asymptomatic APA), and they often lead to an incorrect suspicion of hemorrhagic diathesis, as well as to an unnecessary disqualification from surgical procedures or withdrawal from a proper antithrombotic prophylaxis during these procedures. The aim of the study was to register venous and/or thrombotic events in a group of patients with asymptomatic APA, diagnosed according to the international guidelines (Myakis et all. 2006). Material and methods. The study group consisted of 25 patients (18 women and 7 men) of the mean age of 46 years (20 – 75 years). Concomitantly 9 of them had other autoimmune disorders (no systemic lupus erythematosus) and 4 had neoplasms. Among risk factors for arterial thrombosis 5 patients had hypercholesterolemia, 5 – hypertension, 4 were smokers and 4 were obese (BMI >30 kg/m2). None of the patients had hereditary thrombophilia (antithrombin, protein C or protein S deficiency, factor V Leiden, prothrombin G20210A mutation, increased activity of factor VIII). Family history of venous thromboembolic disease has been noticed in 4 patients and of arterial thrombosis in 6 patients. The observation lasted for 3 to 127 months (mean 35 months). Results. The number and percent of patients (n = 25) with abnormal results 997337of different laboratory diagnostic assays for APA: aPTT-activated partial thromboplastin time; dRVVT - diluted Russell viper venom time; ACA-anticardiolipin antibodies; β2-GPI – anti-β2–glycoprotein I antibodies Abnormal results of more than one assay in any combination were found in 16 patients, lupus anticoagulant alone in 9 patients. Only 4 out of 25 patients have taken aspirin - 75mg daily, in the other 4 a prophylactic dose of low molecular weight heparin was administered temporarily because of surgery. During observation time no venous or arterial thrombotic events occurred in the study group. On a basis of a limited number of previous studies that predominantly included systemic lupus erythematosus patients, aPL-positive patients without previous thrombosis have a 0% to 3.8% annual incident thrombosis risk (Barbhaiya M, Erksan D. Curr Rheumatol Rep. 2011; 13: 59–69). In two patients asymptomatic APA disappeared. Conclusion: independently of the type and quantity of asymptomatic antiphospholipid antibodies, there were no venous or arterial thromboembolic events in the group of patients observed for meanly 35 months. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Isolated IgA Anti-β2 Glycoprotein I Antibodies in Patients with Clinical Criteria for Antiphospholipid Syndrome

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Raquel Ruiz-García ◽  
Manuel Serrano ◽  
José Ángel Martínez-Flores ◽  
Sergio Mora ◽  
Luis Morillas ◽  
...  
Keyword(s):  
Autoimmune Disease ◽  
Antiphospholipid Syndrome ◽  
Antiphospholipid Antibodies ◽  
Arterial Thrombosis ◽  
Clinical Manifestations ◽  
Diagnostic Systems ◽  
Clinical Criteria ◽  
Glycoprotein I ◽  
Standardized Diagnostic ◽  
Disease Present

Seronegative antiphospholipid syndrome (SNAPS) is an autoimmune disease present in patients with clinical manifestations highly suggestive of Antiphospholipid Syndrome (APS) but with persistently negative consensus antiphospholipid antibodies (a-PL). IgA anti-β2 Glycoprotein I (aB2-GPI) antibodies are associated with APS. However, they are not currently considered to be laboratory criteria due to the heterogeneity of published works and the use of poor standardized diagnostic systems. We have aimed to assess aPL antibodies in a group of patients with clinical manifestations of APS (C-APS) to evaluate the importance of the presence of IgA aB2GPI antibodies in APS and its relation with other aPL antibodies. Only 14% of patients with C-APS were positive for any consensus antibody, whereas the presence of isolated IgA aB2GPI antibodies was found in 22% of C-APS patients. In patients with arterial thrombosis IgA aB2GPI, antibodies were the only aPL antibodies present. Serologic profile in primary APS (PAPS) is different from systemic autoimmune disorders associated APS (SAD-APS). IgA aB2GPI antibodies are more prevalent in PAPS and IgG aB2GPI antibodies are predominant in SAD-APS. The analysis of IgA aB2GPI antibodies in patients with clinical manifestations of PAPS might avoid underdiagnosed patients and provide a better diagnosis in patients with SAD-APS. Laboratory consensus criteria might consider including analysis of IgA aB2GPI for APS diagnosis.

scholarly journals AB0408 DOES THE PRESENCE IN THE SERUM OF ANTIPHOSPHOLIPID ANTIBODIES CORRELATE WITH SPECIFIC/NON SPECIFIC CAPILLAROSCOPIC ABNORMALITIES?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1504.2-1504
Author(s):  
G. Ferrari ◽  
S. Paolino ◽  
A. Sulli ◽  
C. Pizzorni ◽  
G. Pacini ◽  
...  
Keyword(s):  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Statistical Correlation ◽  
Systemic Autoimmune Disease ◽  
Research Support ◽  
Vascular Thrombosis ◽  
Pregnancy Morbidity ◽  
Significant Difference ◽  
Cardiolipin Antibodies

Background:Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by specific vascular and obstetric manifestations and by antiphospholipid antibodies (aPL) positivity [1].To date, little is known regarding nailfold videocapillaroscopy (NVC) alterations in APS patients and in asymptomatic aPL-carriers, non-specific abnormalities being the most frequently reported [2,3,4].Objectives:To retrospectively analyze NVC alterations in APS patients and in asymptomatic aPL-carriers and to correlate NCV alterations with both clinical manifestations and serum aPL profile.Methods:Thirty-five aPL positive patients having received at least one NCV investigation (mean age 47 years, range 16-81, 31 female and 4 male) were retrospectively included in the study. For each patient complete medical history was collected with a particular attention to past vascular thrombosis and pregnancy morbidity. Patients were classified as affected by APS according to the updated Sapporo classification criteria [5]. Lupus anticoagulant (LAC), IgM and IgG anti-cardiolipin antibodies (ACL) and IgM and IgG anti-Beta2 Glycoprotein 1 (anti-B2GP1) were assessed in each patient according to the recommended procedures [5]. NCV parameters were analyzed in each patient, with a particular interest to hemorrhages or nailfold bed-parallel hemosiderin deposits (“comb-like”hemorrhages) presence [2,6]. Statistical analysis was performed by parametric and non-parametric tests.Results:Seventeen patients (mean age 49 years, range 16-81 years) were asymptomatic aPL-carriers and 18 (mean age 46 years, range 26-71 years) were affected by APS. Within APS patients, 16 had a history of vascular thrombosis and 2 had pregnancy morbidity; in 6 patients APS was secondary to other autoimmune rheumatologic conditions (3 to Systemic Lupus Erythematosus, 2 to vasculitides and 1 to Mixed Connective Tissue Disease).Among the total number of aPL-carriers and APS patients six patients showed a normal NVC pattern, 24 patients had non-specific NVC abmormalities and 5 patients had a “scleroderma-like” pattern. Interestingly, NCV microhemorrhages were significantly more frequent in APS patients than in asymptomatic aPL-carriers, both in score and in absolute (p=0.05 andp=0.04, respectively). Particularly, in APS patients “comb-like”hemorrhages had a statistically significant higher prevalence than isolated hemorrhages (p=0.03). Dilated capillaries score was significantly higher in APS patients than in asymptomatic aPL-carriers (p=0.01).Not any statistically significant difference was observed regarding other capillary parameters (score of giant capillaries, loss of capillaries, or anormal shpaes, i.e. angiogenesis). Not any statistical correlation was observed between NVC parameters and different aPL profile.Conclusion:The study shows that the total number of microhemorrhages and in particular the“comb-like”subtype, are significantly the most frequent specific abnormalities in APL patients when compared to asymptomatic aPL carriers. The presence of the “scleroderma like” NVC pattern may suggest a concomitant overlap syndrome. Not any correlation was found between aPL profile and other NVC parameters. Further studies need to develop a more specific APS NVC pattern for APS patients.References:[1]Tektonidou MG, et al RMD Open 2019; 5(1);[2]Cutolo M, Elsevier 2010, pp141-143;[3]Candela M, et al.1998:444-9;[4]Aslanidis S, et al. Clin Exp Rheumatol 2011, 29:307-9;[5]Miyakis S, et al. J Thromb Haemost 2006, 4:295–306;[6]Cutolo M, et al Best Pract Res Clin Rheumatol 2008, 22:1093-108Disclosure of Interests: :Giorgia Ferrari: None declared, Sabrina Paolino: None declared, Alberto Sulli Grant/research support from: Laboratori Baldacci, Carmen Pizzorni: None declared, Greta Pacini: None declared, Emanuele Gotelli: None declared, Adriano Lercara: None declared, Vanessa Smith Grant/research support from: The affiliated company received grants from Research Foundation - Flanders (FWO), Belgian Fund for Scientific Research in Rheumatic diseases (FWRO), Boehringer Ingelheim Pharma GmbH & Co and Janssen-Cilag NV, Consultant of: Boehringer-Ingelheim Pharma GmbH & Co, Speakers bureau: Actelion Pharmaceuticals Ltd, Boehringer-Ingelheim Pharma GmbH & Co and UCB Biopharma Sprl, Maurizio Cutolo Grant/research support from: Bristol-Myers Squibb, Actelion, Celgene, Consultant of: Bristol-Myers Squibb, Speakers bureau: Sigma-Alpha

scholarly journals Antiphospholipid syndrome accompanying systemic lupus erythematosus

2002 ◽  
Vol 55 (3-4) ◽  
pp. 89-96 ◽  
Author(s):  
Gorana Mitic
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Clinical Manifestations ◽  
Spontaneous Abortions ◽  
Systemic Lupus ◽  
Recurrent Spontaneous Abortions ◽  
Thrombotic Complications

The aim of the study was the assessment of the prevalence of antiphospholipid syndrome (APS) in patients with systemic lupus erythematosus (SLE). 72 patients with SLE had been investigated, 66 females and six males, aged 17 to 70 years, average 37,03. The presence of APA was determined using both ELISA assay for antiphospholipid antibodies ASSERACHROM APA by Diagnostica Stago and clotting tests for lupus anticoagulant: activated partial thromboplastin time (aPTT), tissue thromboplastin inhibition test (TTI) and dilute Russell viper venom time (dRVVT). Antiphospholipid antibodies have been found in 24 patients (33.44%), 10 of them were. with positive lupus anticoagulant tests, 6 of them were with positive ELISA test, while 8 of them had positive coagulation and immunological tests. Clinical manifestations that could be related to antiphospholipid syndrome were present in 22 patients (30.5%). The most common were thrombotic complications in 16 patients (22.25), recurrent spontaneous abortions in 7 patients (9.7%) and thrombocytopenia in 1 patient (1.39%). Presence of antiphospholipid syndrome was determined in 15 patients (20.83%). We can conclude that there is a significant correlation between presence of antiphospholipid antibodies and both thrombotic events and recurrent spontaneous abortions in SLE patients. Occurrence of thrombotic complications is in direct correlation with the level of antiphospholipid antibodies.

Sign in / Sign up

Export Citation Format

Share Document