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 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

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

Acquired activated protein C resistance is associated with lupus anticoagulants and thrombotic events in pediatric patients with systemic lupus erythematosus

Blood ◽  
2001 ◽  
Vol 97 (4) ◽  
pp. 844-849 ◽  
Author(s):  
Christoph Male ◽  
Lesley Mitchell ◽  
James Julian ◽  
Patricia Vegh ◽  
Penny Joshua ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Protein C ◽  
Pediatric Patients ◽  
Activated Protein C ◽  
Factor V Leiden ◽  
Protein S ◽  
Activated Protein C Resistance ◽  
Systemic Lupus ◽  
Lupus Anticoagulants

Abstract Acquired activated protein C resistance (APCR) has been hypothesized as a possible mechanism by which antiphospholipid antibodies (APLAs) cause thrombotic events (TEs). However, available evidence for an association of acquired APCR with APLAs is limited. More importantly, an association of acquired APCR with TEs has not been demonstrated. The objective of the study was to determine, in pediatric patients with systemic lupus erythematosus (SLE), whether (1) acquired APCR is associated with the presence of APLAs, (2) APCR is associated with TEs, and (3) there is an interaction between APCR and APLAs in association with TEs. A cross-sectional cohort study of 59 consecutive, nonselected children with SLE was conducted. Primary clinical outcomes were symptomatic TEs, confirmed by objective radiographic tests. Laboratory testing included lupus anticoagulants (LAs), anticardiolipin antibodies (ACLAs), APC ratio, protein S, protein C, and factor V Leiden. The results revealed that TEs occurred in 10 (17%) of 59 patients. Acquired APCR was present in 18 (31%) of 58 patients. Acquired APCR was significantly associated with the presence of LAs but not ACLAs. Acquired APCR was also significantly associated with TEs. There was significant interaction between APCR and LAs in the association with TEs. Presence of both APCR and LAs was associated with the highest risk of a TE. Protein S and protein C concentrations were not associated with the presence of APLAs, APCR, or TEs. Presence of acquired APCR is a marker identifying LA-positive patients at high risk of TEs. Acquired APCR may reflect interference of LAs with the protein C pathway that may represent a mechanism of LA-associated TEs.

Expression of the major rheumatoid factor cross-reactive idiotype in pediatric patients with systemic lupus erythematosus

1991 ◽  
Vol 60 (2) ◽  
pp. 232-243 ◽  
Author(s):  
Vincent R. Bonagura ◽  
Norman T. Ilowite ◽  
Lynda Hatam ◽  
David J. Valacer ◽  
Josiah F. Wedgwood
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Rheumatoid Factor ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
Systemic 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.

Pulmonary hemorrhage in pediatric patients with systemic lupus erythematosus

1986 ◽  
Vol 108 (4) ◽  
pp. 576-579 ◽  
Author(s):  
Robert W. Miller ◽  
Jose R. Salcedo ◽  
Robert J. Fink ◽  
Thomas M. Murphy ◽  
Daniel B. Magilavy
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
Pulmonary Hemorrhage ◽  
Systemic Lupus

Performance of 2019 EULAR/ACR classification criteria for Systemic Lupus Erythematosus in a pediatric population – a multicenter study

Rheumatology ◽  
2021 ◽  
Author(s):  
Yoel Levinsky ◽  
Mor Broide ◽  
Shelly Kagan ◽  
Ori Goldberg ◽  
Oded Scheuerman ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Multicenter Study ◽  
Lupus Erythematosus ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Laboratory Data ◽  
Disease Onset ◽  
Classification Criteria ◽  
Systemic Lupus ◽  
Juvenile Sle

Abstract Objectives The "European League Against Rheumatism" and "American College of Rheumatology" 2019 (EULAR/ACR-19) criteria for the diagnosis of Systemic Lupus Erythematosus (SLE) were recently published, with the stated goal of maintaining the level of sensitivity and raising the level of specificity for classification of SLE in adults. The aim of this study is to examine their application to juvenile SLE (jSLE) patients. Methods In this multicenter study the charts of jSLE patients from three tertiary medical centers were reviewed and compared to patients with non-jSLE diagnosis. Pediatric rheumatologists, blinded to the original diagnosis, reviewed and diagnosed all cases. Pediatric patients' clinical and laboratory data were retrospectively extracted and then examined with regard to how they met the new and old criteria. Results Included were 225 patients (112 jSLE, 113 non-SLE). When applied to juvenile SLE classification, the sensitivity of the new EULAR/ACR-19 criteria was 0.96 (0.9-.0.99) and the specificity was 0.89 (0.82-0.94). These were comparable to the Systemic Lupus International Collaborating Clinics (SLICC) criteria. . The sensitivity of the EULAR/ACR-19 criteria improves over time and was 0.83 twelve months following disease onset, reaching 0.96 after longer than 24 months. Conclusion Among a cohort of jSLE patients, sensitivity of the new EULAR/ACR-19 criteria was found to be high and specificity may have improved slightly compared to the SLICC-12 criteria. We support the use of the new classification criteria for pediatric patients in future jSLE studies, but it should be noted that its specificity is lower than for adults.

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