scholarly journals Evidence of a distinct group of Black African patients with systemic lupus erythematosus

2018 ◽  
Vol 3 (5) ◽  
pp. e000697 ◽  
Author(s):  
Elopy N Sibanda ◽  
Margo Chase-Topping ◽  
Lorraine T Pfavayi ◽  
Mark E J Woolhouse ◽  
Francisca Mutapi
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Trna Synthetase ◽  
High Morbidity ◽  
Soluble Substance ◽  
Systemic Lupus ◽  
Predictive Values ◽  
Black African ◽  
African Populations ◽  
African Patients

BackgroundThe autoimmune disease systemic lupus erythematosus (SLE) occurs more frequently in patients of African descent with high morbidity and mortality. Current SLE diagnostic criteria including antinuclear antibody (ANA) reactivity are derived largely from non-African populations. This study characterises ANA reactivity patterns and relates them to SLE clinical presentation in Black African patients.MethodsSera from Black participants (61 patients with SLE and 100 controls) aged 1–81 years were analysed for reactivity against the antigens: uridine 1-ribonuclear protein, Smith uridine-1-5 ribonuclear protein antigen, soluble substance-A, recombinant Ro-52, soluble substance-B, Scl-70, cytoplasmic histidyl-tRNA synthetase antigen, proliferating cell nuclear antigen (PCNA), nucleosomes, ribonuclear P-protein, antimitochondrial antibody M2 (AMA-M2), histones, double-stranded DNA (dsDNA), centromere protein B and polymyositis–sclerosis overlap antigen.FindingsA significantly higher proportion (97%) of the 61 patients with SLE had detectable autoantibody reactivity compared with 15% of the 100 controls (p<0.001). The highest frequencies of autoantibody reactivity in patients with SLE were against the dsDNA antigen (41%) and PCNA (54%). Anti-PCNA and anti-dsDNA reactivity were mutually exclusive (p<0.001) giving rise to two distinct groups of Black African patients with SLE. The first group (n=25) had reactivity profiles consistent with international standard SLE definitions, including anti-dsDNA reactivity, and was 13 times more likely to present with joint symptoms. The larger, second group (n=34), characterised by anti-PCNA and anti-AMA-M2 reactivity, was nine times more likely to present with only cutaneous symptoms.InterpretationOur study demonstrates a need to extend autoantibody panels to include anti-PCNA in the diagnostic process of Black African patients and further refine the predictive values of the reactivity to different antigens to differentiate SLE syndromes in African populations.

scholarly journals FRI0152 PULMONARY HYPERTENSION IN NEWLY DIAGNOSED SPANISH PATIENTS WITH SYSTEMIC LUPUS ERYTHEMATOSUS: DATA FROM THE RELES COHORT

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 660.2-660
Author(s):  
J. Álvarez Troncoso ◽  
Á. Robles Marhuenda ◽  
F. Mitjavila Villero ◽  
F. J. García Hernández ◽  
A. Marín Ballvé ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Pulmonary Hypertension ◽  
Lupus Erythematosus ◽  
Multivariable Analysis ◽  
Systolic Pressure ◽  
High Morbidity ◽  
Inception Cohort ◽  
Systemic Lupus ◽  
Factors Associated ◽  
Pulmonary Arterial

Background:Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiorgan involvement. Pulmonary hypertension (PH) is an uncommon manifestation with high morbidity and mortality whose characteristics, prevalence and evolution in SLE are not completely defined.Objectives:Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to to identify the factors associated with pulmonary hypertension (PH) in systemic lupus erythematosus (SLE).Methods:Prospective observational study on a multicenter Spanish inception cohort. Patients with SLE, diagnosed by the American College of Rheumatology (ACR) criteria, since January 2009, who had at least one transthoracic echocardiogram (TTE) performed were selected. Demographic data, diagnostic criteria, follow-ups, treatments and SLEDAI were analyzed.Results:Of 289 patients diagnosed with SLE with TTE performed, 15 (5.2%) patients were identified to have PH. Mean age was 56,9±7,7 years, of which 93,3% (14) were women and 80% (12) Caucasian. The ACR score at diagnosis was 4.66. Mean SLEDAI was 15. Only 5 patients had dyspnea at the time of diagnosis. Mean pulmonary arterial systolic pressure was 49.2±5.6 mmHg. Among the PH, 4 patients had pericarditis (26.6%), 3 (20%) valvulopathies (1 antiphospholipid syndrome), 1 patient pulmonary embolism and 1 shrinking lung. Multivariable analysis indicated that pericarditis (odds ratio (OR)=2.53), and valvulopathies (OR 8.96) were independently associated with the development of PH in SLE. Having PH was associated with older age at diagnosis (p<0.001), more dyspnea (p<0.001), higher ESR (p=0.007), more serositis (p<0.001), higher SLEDAI (p=0.011), higher SLICC (p <0.001), higher number of admissions (p=0.006) and higher mortality (p=0.003).Conclusion:PH in SLE is a serious comorbidity with high mortality. In the RELES cohort it was associated with increased disease activity, pericarditis and valvulopathies. Performing TTE in patients with SLE may favor early diagnosis and treatment.References:[1]Kim JS, Kim D, Joo YB, et al. Factors associated with development and mortality of pulmonary hypertension in systemic lupus erythematosus patients.Lupus. 2018;27(11):1769–1777.[2]Bazan IS, Mensah KA, Rudkovskaia AA, et al. Pulmonary arterial hypertension in the setting of scleroderma is different than in the setting of lupus: A review.Respir Med. 2018;134:42–46.Disclosure of Interests:Jorge Álvarez Troncoso: None declared, Ángel Robles Marhuenda: None declared, Francesca Mitjavila Villero: None declared, Francisco José García Hernández: None declared, Adela Marín Ballvé: None declared, Antoni Castro Consultant of: Actelion pharmaceuticals, GSK, MSD., Gonzalo Salvador Cervelló: None declared, Eva Fonseca: None declared, Isabel Perales Fraile: None declared, Guillermo Ruiz-Irastorza: None declared

scholarly journals EMBRACE: Phase 3/4, Randomized, 52‐Week Study of Belimumab Efficacy and Safety in Patients of Black African Ancestry With Systemic Lupus Erythematosus

2021 ◽  
Author(s):  
Ellen Ginzler ◽  
Luiz Sergio Guedes Barbosa ◽  
David D’Cruz ◽  
Richard Furie ◽  
Kathleen Maksimowicz‐McKinnon ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
African Ancestry ◽  
Efficacy And Safety ◽  
Systemic Lupus ◽  
Phase 3 ◽  
Black African

P041 Systemic Lupus Erythematosus in Algerian Children: clinical, immunological profile and prognosis

Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
O Gacem ◽  
L Labboun ◽  
N Mansouri ◽  
M Gherbi ◽  
Z Zeroual ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Odds Ratio ◽  
Protective Factor ◽  
High Morbidity ◽  
Antiphospholipid Antibody ◽  
Systemic Lupus ◽  
Significant Difference ◽  
The Mean

Abstract Background Pediatric Systemic Lupus Erythematosus (pSLE) is a chronic mutisystemic autoimmune disease with complex clinical manifestations whose diagnosis is not always easy and the course is generally severe and the treatment is not very well codified and often extrapolated from that of adults. This study aims to describe the clinical, immunological, therapeutic characteristics and short outcome of systemic lupus erythematosus in Algerian children. Methods This was a prospective, multicentre and descriptive study 36 months (January 2015 - December 2018) at the department of Pediatrics of University Hospital Nefissa Hamoud ex Parnet Algiers. Children less than16 years of age fulfilling the American College of Rheumatology SLE criteria were included. Disease activity estimated by Systemic Lupus Erythematosus Disease Activity index (SLEDAI) whose use has been validated in children and damage index based on Systemic Lupus International Collaborating Clinics (SLICC) score were determined. Results Eighty-three (83) patients were studied. Female: male ratio was1:49. Mean ages at lupus onset and diagnosis were respectively: 10, 12 ± 3, 88 and 11, 3 ± 3, 62 years. All patients had skin involvement while constitutional signs including fever and asthenia were observed in (98.8%). Rheumatological, renal, neuropsychiatric, cardiac, hepato-digestive, pleuropulmonary and ocular disorders were observed respectively: 65, 1%, 44, 6%, 41%, 27, 7%, 41%, 19, 3% and 7, 2%. All patients were positive for antinuclear antibodies. Anti-double-stranded DNA (75%) was the most frequently observed autoantibody profile. Antiphospholipid antibody positivity was noted in 52% whereas hypocomplementemia in fractions C3, C4 was observed in 55% and 56% respectively. In our study, the severe forms were more frequent (83%) than the mild ones (17%) with a significant difference (P = &lt; 10–6). Overall, the mean SLEDAI at disease onset was 22.11 ± 11.87 with high activity ≥ 20 in 59% of cases. The mean damage score was 1.8 ± 2.045 (interquartile range 0–8). Among induction drugs, oral corticosteroids were the most frequently used (92%), and in a third of cases intravenously at high doses in combination with immunosuppressive therapy. In induction therapy, cyclophosphamide (CYC) was the most used drug (23%) compared with mycophenolate mofetil (MMF) (14%). Unlike the maintenance phase where MMF observed an increase (28%) vs (8%) CYC. The use of MMF was correlated with severe lupus nephritis with a significantly effective difference in the decrease in SLEDAI (P = 0.0001). The use of hydroxychloroquine (HCQ) was observed in 81% in induction and 89% in maintenance treatment. The correlation of HCQ use with survival was significantly positive (P = 0.04). Indeed, adherence to treatments and essentially HCQ was a protective factor, its odds ratio is &lt; 1 with a significant p-value, [OR 0.016 95% CI (0.001–0.353)]. Mortality was estimated at 11%. Multivariable regression analysis showed that the neurological involvement (odds ratio = 6,093 95% confidence interval ((1,1 8 0 ∼ 31 446)) and macrophage activation syndrome were associated with a high risk of mortality. Conclusion we report a series of pSLE characterized by great clinical and biological heterogeneity. It follows a severe course of the disease with high disease activity at the diagnosis and therefore leads to high morbidity and mortality. However, these results must be confirmed by other pediatric studies which could form the basis of a diagnostic and therapeutic approach more adapted for children. Keywords Algeria, Child, Clinical features, Disease activity, lupus

scholarly journals A Case of Systemic Lupus Erythematosus Presenting as Guillain-Barré Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Helen Chioma Okoh ◽  
Sandeep Singh Lubana ◽  
Spencer Langevin ◽  
Susan Sanelli-Russo ◽  
Adriana Abrudescu
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Disease ◽  
Complete Recovery ◽  
Multiple Organ ◽  
Guillain Barre Syndrome ◽  
High Morbidity ◽  
Systemic Lupus ◽  
Guillain Barré Syndrome ◽  
Guillain Barré

Systemic lupus erythematosus (SLE) is an autoimmune systemic disease with multiple organ involvement with high morbidity and mortality rate. Among the severe potential fatal complications are those of the central and peripheral nervous system which usually develop during the course of the disease and very rarely from the outset of the disease. We are reporting a rare case of Miller-Fisher (MFS) variant of Guillain-Barré syndrome (GBS) as the first manifestation of SLE in a 41-year-old female who progressed to flaccid paralysis with no neurological improvement with initial immunosuppressive therapy, plasmapheresis, and first cycle of intravenous immunoglobulin (IVIG) but with remarkable and complete recovery after the second 5-day course of IVIG.

Assessment of Coronary Risk Based on Cumulative Exposure to Lipids in Systemic Lupus Erythematosus

2013 ◽  
Vol 40 (12) ◽  
pp. 2006-2014 ◽  
Author(s):  
Mandana Nikpour ◽  
Dafna D. Gladman ◽  
Dominique Ibanez ◽  
Paula J. Harvey ◽  
Murray B. Urowitz
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Density Lipoprotein ◽  
Coronary Event ◽  
Lipoprotein Cholesterol ◽  
Cumulative Exposure ◽  
Coronary Risk ◽  
Systemic Lupus ◽  
Contingency Table Analysis ◽  
Predictive Values

Objective.To quantify the independent role of each of low-density lipoprotein cholesterol (LDL-C), total cholesterol:high-density lipoprotein cholesterol ratio (TC:HDL-C), triglyceride (TG) level, and HDL-C as a marker of coronary risk in systemic lupus erythematosus (SLE).Methods.Patients with lipid measurements taken before a coronary event (or last clinic visit) were included. Mean and time-adjusted mean (TAM) levels were calculated for each lipid variable in each patient. Time-dependent proportional hazards regression models were used to quantify the risk of coronary event [myocardial infarction (MI) or angina], after adjustment for age.Results.Among 384 patients, over a mean (SD) followup of 3.81 (2.58) years, there were 21 “first” coronary events (6 MI, 15 angina). Mean and TAM LDL-C (HR 1.83, 95% CI 1.19–2.81, p = 0.006), TC:HDL ratio (HR 1.43, 95% CI 1.02–2.00, p = 0.04), and TG (HR 2.11, 95% CI 1.32–3.39, p = 0.0019) were predictive of coronary event at subsequent visits. In contingency table analysis, TAM LDL-C cutpoint of 2.0 mmol/l had a sensitivity and negative predictive value for coronary event of 85.7% (95% CI 63.7–97.0) and 93.9% (95% CI 83.1–98.7), respectively. However, at this cutpoint the specificity was only 12.7% (95% CI 9.4–16.5).Conclusion.This study links LDL-C, TC:HDL-C ratio, and TG to coronary risk in patients with SLE and quantifies the magnitude of this risk. SLE-specific risk assessment levels for lipids may be selected to optimize positive or negative predictive values.

scholarly journals Invasive Group B Streptococcal Disease in Two Pediatric Patients with Systemic Lupus Erythematosus

2013 ◽  
Vol 2013 ◽  
pp. 1-3
Author(s):  
Dongning Wu ◽  
Kenneth Bromberg ◽  
Roberto Jodorkovsky
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
High Morbidity ◽  
Systemic Lupus ◽  
Invasive Group ◽  
Pediatric Age Group ◽  
Immunosuppressive Medication ◽  
Group B Streptococcal Disease ◽  
Group B

Systemic lupus erythematosus (SLE) is an autoimmune disease associated with high morbidity and mortality, often caused by infection. We report two patients with SLE who were treated with steroids and immunosuppressive medication and then developed invasive Group BStreptococcus(GBS) infections. While GBS infection is rare in the nonneonatal pediatric age group, GBS should be considered when treating SLE patients presenting with signs of infection.

scholarly journals Rituximab for Remission Induction and Maintenance in Refractory Systemic Lupus Erythematosus

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Fabio Bonilla-Abadía ◽  
Nicolás Coronel Restrepo ◽  
Gabriel J. Tobón ◽  
Andrés F. Echeverri ◽  
Evelyn Muñoz-Buitrón ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Conventional Treatment ◽  
Drug Dose ◽  
Immunosuppressive Drug ◽  
Remission Induction ◽  
High Morbidity ◽  
Systemic Lupus ◽  
Maintenance Of Remission ◽  
Inflammatory Autoimmune Disease

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease with high morbidity if untreated. Sometimes, despite aggressive treatments, the disease remains active with cumulative organic damage. We conducted a retrospective and descriptive observational study of patients with SLE refractory to conventional treatment who were treated with rituximab (RTX) as remission induction therapy and maintenance. There was a significant reduction in the conventional immunosuppressive drug dose and the number of relapses of disease. RTX appeared to be effective and safe for the induction and maintenance of remission in patient with SLE refractory to conventional treatment.

scholarly journals Bacteremia in female Chinese patients with systemic lupus erythematosus: a case-control study

2017 ◽  
Vol 11 (05) ◽  
pp. 393-398 ◽  
Author(s):  
Fangru Chen ◽  
Fei Hao ◽  
Qiquan Chen ◽  
Tian Qian ◽  
Yan Chen ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Activity Index ◽  
Laboratory Data ◽  
Critical Factor ◽  
Case Control ◽  
University Hospital ◽  
Chinese Patients ◽  
High Morbidity ◽  
Systemic Lupus

Introduction: Bacteremia is a common complication in systemic lupus erythematosus (SLE) patients, causing high morbidity and mortality. We investigated characteristics, pathogens, and sites of infection using a cohort of 64 female adults from a single university hospital in China. Methodology: SLE patients who had at least one episode of bacteremia (n = 16) were compared with non-bacteremia SLE patients (n = 48) in a case-control fashion, matching for age at SLE diagnosis and time of admission. Demographic characteristics, clinical and laboratory data, and bacteriologic examinations were collected and reviewed. Results: A series of parameters were found to be significantly different between controls and cases at bacteremia diagnosis, including an SLE disease activity index, multiple major organ involvement (> 2), active renal disease, leukocytes, neutrophils, 24-hour urine protein, erythrocyte sedimentation rate (ESR), aspartate aminotransferase (AST), creatinine, hemoglobin, lymphocyte, platelets, and albumin. Eighteen episodes of bacteremia were analyzed, with Escherichia coli and Staphylococcus aureus being the most frequent isolates. Additionally, Listeria monocytogenes, Rhodotorula mucilaginosa, and Salmonella choleraesuis, which were very rare in the general population, were isolated from the bloodstreams of the cases. Apart from bacteremia without focus, respiratory tract, gastrointestinal tract, urinary tract, skin, and soft tissue were the major origins of infection. Conclusions: The present study depicts the nature of a cohort of female Chinese SLE patients with bacteremia, revealing that bacteremia is a critical factor contributing to the aggravation of SLE. Our findings provide useful information regarding the control and prevention of bacteremia in female SLE patients in China.

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