scholarly journals Atypical Presentations of Systemic Lupus Erythematosus by Cerebral Venous Thrombosis and Generalized Lymphadenopathy without Antiphospholipid Antibodies: Case Report

Author(s):  
Xinye Serena Wang ◽  
William Magnuson ◽  
Pearl Behl ◽  
Dmitrii Koval

Abstract Introduction: Atypical presentations of systemic lupus erythematosus SLE) with features outside of the 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria can make the diagnosis of SLE elusive. Case Presentation: We describe a case of a healthy 31-year old female who presented with syncope preceded by progressive headache, serositis, and generalized lymphadenopathy. CT-head and MR-venogram confirmed a cerebral venous thrombosis (CVT). Subsequent workup revealed high titers of antinuclear (ANA), anti-double stranded DNA (anti-dsDNA), anti-Smith (anti-Sm) antibodies, as well as low complement levels, lymphopenia and neutropenia. Diagnosis of SLE was confirmed by SLICC classification criteria. Antiphospholipid antibodies (APLA) were negative. While in hospital, she sustained a seizure secondary to the CVT. Conclusion: We discuss considerations for atypical SLE presentations by CVT (without APLA syndrome) and generalized lymphadenopathy, and the nuances of SLE diagnosis using existing classification criteria.

2009 ◽  
Vol 36 (6) ◽  
pp. 1195-1199 ◽  
Author(s):  
ADRIANA DANOWSKI ◽  
MARIO NEWTON LEITÃO de AZEVEDO ◽  
JOSE ANGELO de SOUZA PAPI ◽  
MICHELLE PETRI

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.


2010 ◽  
Vol 38 (1) ◽  
pp. 64-68 ◽  
Author(s):  
TARANEH MEHRANI ◽  
MICHELLE PETRI

Objective.IgA isotypes of anticardiolipin and anti-ß2 glycoprotein I (anti-ß2-GPI) are omitted from the revised antiphospholipid syndrome (APS) classification criteria. Multiple studies have found a high prevalence of IgA anti-ß2-GPI in systemic lupus erythematosus (SLE). We determined the frequency and associations of IgA anti-ß2-GPI in a cohort of patients with SLE.Methods.Anti-ß2-GPI was measured in 796 patients with SLE (93% women, 53% white, 38% African American, mean age 45 yrs). IgA anti-ß2-GPI (> 20 phospholipid units) was found in 20%. Using a cohort database, associations with cumulative thrombotic and other manifestations were determined.Results.Of patients with SLE who demonstrated IgA anti-ß2-GPI positivity, about 6% had transient ischemic attack (p = 0.070), 4% had superficial thrombophlebitis (p = 0.647), 20% had deep venous thrombosis (p = 0.003), 4% had other venous thrombosis (p = 0.827), 12% had stroke (p = 0.050), and 1% had myocardial infarction (p = 0.397).Conclusion.IgG anti-ß2-GPI has the strongest association with thrombosis in SLE. However, IgA anti-ß2-GPI was more strongly associated with deep venous thrombosis and with stroke than was IgM. These results indicate that assessment of IgA anti-ß2-GPI is associated with thrombosis in SLE, and that the classification criteria for APS should be revised to include IgA anti-ß2-GPI in patients with SLE.


Lupus ◽  
1997 ◽  
Vol 6 (5) ◽  
pp. 467-473 ◽  
Author(s):  
DG Wahl ◽  
F. Guillemin ◽  
E. de Maistre ◽  
C. Perret ◽  
T. Lecompte ◽  
...  

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Dana DiRenzo ◽  
Zsuzsanna H. McMahan ◽  
Naman S. Desai ◽  
Rebecca Manno ◽  
Michelle Petri

The differential diagnosis for a focal brain lesion in a patient with systemic lupus erythematosus (SLE) is broad and includes infection, malignancy, and vascular and inflammatory etiologies. One rarely considered vascular pathology is cerebral venous thrombosis (CVT), which is often associated with a delay in diagnosis because of variable presentation and rare incidence. We present the case of a young woman with a new discrete brain lesion that appeared in the context of highly active SLE and was ultimately diagnosed with a CVT. We provide a literature review for diagnosis and management of cerebral venous thrombosis, a potentially serious complication of untreated systemic lupus erythematosus.


2019 ◽  
Vol 8 (2) ◽  
pp. 101-102
Author(s):  
Miguel Garcia‐Grimshaw ◽  
Amado Jimenez‐Ruiz ◽  
Juan Andres Calderon‐Martinez

1996 ◽  
Vol 76 (04) ◽  
pp. 514-517 ◽  
Author(s):  
R Fijnheer ◽  
D A Horbach ◽  
R C J M Donders ◽  
H Vilé ◽  
E v Oort ◽  
...  

SummaryThromboembolic complications are frequently observed in patients with systemic lupus erythematosus (SLE). Significant associations have been reported between these complications and the presence of antiphospholipid antibodies, notably the lupus anticoagulant and anti-cardiolipin antibodies. Factor V Leiden is a genetic disorder associated with an increased risk of venous thrombosis. We studied these factors in 173 patients with SLE in relation to both arterial and venous thrombosis. The frequency of factor V Leiden in SLE patients is comparable to that in the Dutch population (5%) and a risk factor for venous thrombosis (odds ratio 4.9; Cl 1.2-19.6), but not for arterial thrombosis. The lupus anticoagulant is a risk factor for both arterial thrombosis (odds ratio 7.1; Cl 2.9-17.4) and venous thrombosis (odds ratio 6.4; Cl 2.7-15.4). From multivariate analysis, both the lupus anticoagulant and factor V Leiden appeared independent risk factors for venous thrombosis.


Lupus ◽  
2020 ◽  
Vol 30 (1) ◽  
pp. 172-174
Author(s):  
María Elena Aguirre-Alastuey ◽  
Silvia Suárez-Díaz ◽  
Francisco Rodríguez-Jerez ◽  
Rubén Coto-Hernández ◽  
Luis Caminal-Montero

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