Hematological involvement in pediatric systemic lupus erythematosus: A multi-center study

Lupus ◽  
2021 ◽  
pp. 096120332110142
Author(s):  
Ümmüşen K Akca ◽  
Ezgi D Batu ◽  
Ayşenur P Kısaarslan ◽  
Hakan Poyrazoğlu ◽  
Nuray A Ayaz ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Systemic Lupus ◽  
Antibody Positivity ◽  
Hematological Manifestations ◽  
Multi Center Study ◽  
Pediatric Sle ◽  
Center Study

Introduction Systemic lupus erythematosus (SLE) may present with features of several systems, including hematological manifestations. In this study, we aimed to evaluate the characteristics of hematological involvement and assess possible associations and correlations in pediatric SLE patients. Method This is a retrospective multi-center study. The medical records of pediatric SLE patients followed between January 2000 and June 2020 were analyzed. All children fulfilled the criteria of the Systemic Lupus International Collaborating Clinics. Results The study included 215 children with SLE, 118 of whom had hematological manifestations. Concomitant renal involvement and low C3 levels were significantly more frequent in patients with hematological involvement ( p = 0.04, p = 0.008, respectively). Also, anti-cardiolipin, anti-beta-2-glycoprotein I (anti-β2 GP1), and anti-Smith (anti-Sm) antibody positivity, and the presence of lupus anticoagulant were more common in the group with hematological findings ( p = 0.001 for anti-cardiolipin antibody positivity and p < 0.001 for the positivity of anti-β2 GP1 antibody, anti-Sm antibody, and lupus anticoagulant). The most common hematologic abnormality was anemia ( n = 88, 74.5%), with autoimmune hemolytic anemia constituting the majority ( n = 40). Corticosteroids followed by IVIG were the mainstay of treatment. In patients resistant to corticosteroid and IVIG treatments, the most preferred drug was rituximab. The presence of antiphospholipid antibodies and high SLEDAI score were independently associated with hematological involvement in multivariate analysis (OR: 0.249; 95%CI: 0.126–0.490; p < 0.001 and OR: 1.136; 95%CI: 1.065–1.212; p < 0.001). Conclusion Hematological abnormalities are frequently encountered in pediatric SLE. Positive antiphospholipid antibodies and high SLEDAI scores were associated with hematological involvement.

Hematological involvement in pediatric systemic lupus erythematosus: A multi-center study

Lupus ◽  
2021 ◽  
pp. 096120332110388
Author(s):  
Ümmüşen Kaya Akca ◽  
Ezgi Deniz Batu ◽  
Ayşenur Pac Kısaarslan ◽  
Hakan Poyrazoğlu ◽  
Nuray Aktay Ayaz ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Renal Involvement ◽  
Systemic Lupus ◽  
Antibody Positivity ◽  
Hematological Manifestations ◽  
Multi Center Study ◽  
Pediatric Sle ◽  
Center Study

Introduction: Systemic lupus erythematosus (SLE) may present with features of several systems, including hematological manifestations. In this study, we aimed to evaluate the characteristics of hematological involvement and assess possible associations and correlations in pediatric SLE patients. Method: This is a retrospective multi-center study. The medical records of pediatric SLE patients followed between January 2000 and June 2020 were analyzed. All children fulfilled the criteria of the Systemic Lupus International Collaborating Clinics. Results: The study included 215 children with SLE, 118 of whom had hematological manifestations. Concomitant renal involvement and low C3 levels were significantly more frequent in patients with hematological involvement ( p = 0.04, p = 0.008, respectively). Also, anti-cardiolipin, anti-beta-2-glycoprotein I (anti-β2 GP1), and anti-Sm antibody positivity, and the presence of lupus anticoagulant were more common in the group with hematological findings ( p = 0.001 for anti-cardiolipin antibody positivity and p < 0.001 for the positivity of anti-β2 GP1 antibody, anti-Sm antibody, and lupus anticoagulant). The most common hematologic abnormality was anemia (n = 88, 74.5%), with autoimmune hemolytic anemia constituting the majority (n = 40). Corticosteroids followed by IVIG were the mainstay of treatment. In patients resistant to corticosteroid and IVIG treatments, the most preferred drug was rituximab. Low levels of C3, high SLEDAI score, high incidence of renal involvement, and positive antiphospholipid antibodies were associated with hematological involvement in the univariate analysis. The presence of antiphospholipid antibodies and high SLEDAI score were independently associated with hematological involvement in multivariate analysis (OR: 4.021; 95% CI: 2.041–7.921; p < 0.001 and OR: 1.136; 95% CI: 1.065–1.212; p < 0.001). Conclusion: Hematological abnormalities are frequently encountered in pediatric SLE. Positive antiphospholipid antibodies and high SLEDAI scores were associated with hematological involvement.

The relationship of antiphospholipid antibodies to cognitive function in patients with systemic lupus erythematosus

1997 ◽  
Vol 3 (4) ◽  
pp. 377-386 ◽  
Author(s):  
SUSAN D. DENBURG ◽  
RAMONA M. CARBOTTE ◽  
JEFFREY S. GINSBERG ◽  
JUDAH A. DENBURG
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Verbal Memory ◽  
Lupus Anticoagulant ◽  
Cognitive Test ◽  
Antiphospholipid Antibody ◽  
Systemic Lupus ◽  
Cross Sectional ◽  
Antibody Positivity ◽  
The Relationship

Objective: To examine the relationship between antiphospholipid antibody positivity (expressed as the lupus anticoagulant) and cognitive dysfunction in patients with systemic lupus erythematosus (SLE). Methods: Cross-sectional comparisons of lupus anticoagulant (LA) positive (N = 39) and negative (N = 79) patients and controls (N = 35) on a cognitive test battery; 22 LA-positive and 53 LA-negative patients who had never experienced neuropsychiatric events (never-NP–SLE) were also compared separately. Results: LA-positive patients were 2 to 3 times more likely than were LA-negative patients to be designated as cognitively impaired. As a group, LA-positive patients, particularly those in the never-NP–SLE group, demonstrated lower performance primarily on tasks of verbal memory, cognitive flexibility, and psychomotor speed. Conclusions: LA positivity is associated with subclinical nervous system compromise, and a pattern of deficits compatible with subcortical involvement, possibly on the basis of ongoing LA-related microthrombotic events or vasculopathy. (JINS, 1997, 3, 377–386.)

scholarly journals Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus

2003 ◽  
Vol 58 (3) ◽  
pp. 157-162 ◽  
Author(s):  
Lúcia Maria de Arruda Campos ◽  
Maria Helena B. Kiss ◽  
Élbio A. D'Amico ◽  
Clóvis Artur Almeida Silva
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Children And Adolescents ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Anticardiolipin Antibodies ◽  
Systemic Lupus ◽  
Severity Scores ◽  
And Behavior

OBJECTIVE: To investigate the frequencies and behavior of antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus. METHODS: Anticardiolipin antibodies were investigated by ELISA and lupus anticoagulant antibodies by the international tests recommended. The antiphospholipid antibodies analyses were performed in frozen samples (mean of 5.3 samples per patient obtained during a mean follow-up period of 3 years and 7 months) and on blood samples collected between January 1997 and November 1998 (mean of 2.5 samples per patient during a 2-year follow-up period). RESULTS: The frequencies of antiphospholipid antibodies (anticardiolipin and lupus anticoagulant) were similar in the samples collected prospectively and in the frozen samples (retrospective study): 63.2% and 75.4% respectively. Positivity for these antibodies fluctuated during the follow-up period and was not associated with any clinical or laboratory parameters of lupus erythematosus, including autoantibodies and also including disease activity and/or severity scores. CONCLUSIONS: The frequencies of antiphospholipid antibodies in children and adolescents with lupus erythematosus were similar to those observed in adults. The positivity fluctuated during the follow-up and was not correlated with clinical and/or laboratory disease parameters.

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.

Factor V Leiden, Antiphospholipid Antibodies and Thrombosis in Systemic Lupus Erythematosus

1996 ◽  
Vol 76 (04) ◽  
pp. 514-517 ◽  
Author(s):  
R Fijnheer ◽  
D A Horbach ◽  
R C J M Donders ◽  
H Vilé ◽  
E v Oort ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Venous Thrombosis ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Odds Ratio ◽  
Arterial Thrombosis ◽  
Lupus Anticoagulant ◽  
Factor V Leiden ◽  
Factor V ◽  
Systemic Lupus

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.

Transient monocular blindness and antiphospholipid antibodies in systemic lupus erythematosus

Neurology ◽  
1998 ◽  
Vol 51 (2) ◽  
pp. 535-540 ◽  
Author(s):  
R. C.J.M. Donders ◽  
L. J. Kappelle ◽  
R. H.W.M. Derksen ◽  
A. Algra ◽  
D. A. Horbach ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Odds Ratio ◽  
Lupus Anticoagulant ◽  
Normal Population ◽  
Livedo Reticularis ◽  
Systemic Lupus ◽  
Transient Monocular Blindness ◽  
Visual Disturbances

Background and Objective: Among patients with systemic lupus erythematosus (SLE), the presence of antiphospholipid antibodies (APA), notably the lupus anticoagulant, and anticardiolipin antibodies (aCL) characterizes a subset of patients with a thrombotic tendency. During the regular follow-up care of patients with SLE, we noticed that many described transient visual disturbances. Because a hypercoagulable state may cause transient monocular blindness (TMB), we determined the frequency of TMB and studied its relation to the presence of APA in patients with SLE.Methods: We asked 175 unselected patients with SLE whether they had transient visual disturbances and reviewed their medical charts. All patients were examined with specific attention to the presence of livedo reticularis. Blood was examined for APA.Results: Visual disturbances were recorded for 136 (78%) patients. According to predefined criteria, the symptoms were diagnosed as TMB for 10 (6%) patients and as visual disturbances associated with migraine for 18 (10%) patients. Five of the 10 patients with TMB had attacks in either eye. The 175 patients with SLE accrued a maximum total of 6,349 patient years in their lifetime. From this, the incidence of TMB can be calculated to be at least 158 per 100,000 per year. Lupus anticoagulant was detected in 3 of 10 patients with TMB and 41 of 165 patients without TMB (odds ratio, 1.3; 95% CI, 0.2 to 6.0). aCLs were found in 5 of 10 patients with TMB and 91 of 165 patients without TMB (odds ratio, 0.8; 95% CI, 0.2 to 3.7).Conclusions: The frequency of TMB among patients with SLE is at least 158 per 100,000 compared with the normal population (14 per 100,000 per year). However, among patients with SLE, no significant relation could be shown between TMB and the presence of APA or livedo reticularis.

scholarly journals Hydroxychloroquine Use Is Associated with Lower Odds of Persistently Positive Antiphospholipid Antibodies and/or Lupus Anticoagulant in Systemic Lupus Erythematosus

2012 ◽  
Vol 40 (1) ◽  
pp. 30-33 ◽  
Author(s):  
ANNA BRODER ◽  
CHAIM PUTTERMAN
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Lupus Anticoagulant ◽  
Care Center ◽  
Tertiary Care ◽  
Active Role ◽  
Outcome Variable ◽  
Systemic Lupus ◽  
Lower Odds

Objective.Antiphospholipid antibodies (aPL) play an active role in the pathogenesis of the antiphospholipid syndrome (APS). Primary prevention in APS may be aimed at decreasing existing elevated aPL levels, or preventing high aPL titers and/or lupus anticoagulant (LAC) from developing in the first place. Hydroxychloroquine (HCQ) has been shown in retrospective studies to decrease aPL titers in laboratory studies, and to decrease thrombosis risk in patients with systemic lupus erythematosus (SLE). We investigated an association between HCQ use and persistent aPL and/or LAC in SLE.Methods.We identified all patients over 21 years old with SLE from an urban tertiary care center who had aPL and LAC measured on at least 2 occasions at least 12 weeks apart. We defined the presence of persistent LAC+ and/or at least 1 aPL ≥ 40 U [immunoglobulin A (IgA), IgG, or IgM] as the main outcome variable.Results.Among 90 patients included in the study, 17 (19%) had persistent LAC+ and/or at least 1 aPL ≥ 40 U. HCQ use was associated with significantly lower odds of having persistent LAC+ and/or aPL ≥ 40 U (OR 0.21, 95% CI 0.05, 0.79, p = 0.02), adjusted for age, ethnicity, and sex.Conclusion.This is the first study to show that HCQ use is associated with lower odds of having persistently positive LAC and/or aPL. Data from this study provide a basis for the design of future prospective studies investigating the role of HCQ in primary and secondary prevention of APS.

The performances of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 classification criteria in pediatric systemic lupus erythematosus

2020 ◽  
pp. jrheum.200871
Author(s):  
Ezgi Deniz Batu ◽  
Ummusen Kaya Akca ◽  
Aysenur Pac Kısaarslan ◽  
Erdal Sağ ◽  
Ferhat Demir ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antibody Test ◽  
Classification Criteria ◽  
Joint Involvement ◽  
Systemic Lupus ◽  
Pediatric Systemic Lupus Erythematosus ◽  
American College Of Rheumatology ◽  
Hematological Manifestations ◽  
Pediatric Sle

Objective Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The ACR (American College of Rheumatology) 1997, SLICC (Systemic Lupus International Collaborating Clinics) 2012, and EULAR (European League Against Rheumatism)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric SLE patients. Methods Pediatric SLE patients (n=262; 80.9% female) were included from three different centers in Turkey. As controls, 174 children (60.9% female) with other diseases who had ANA (antinuclear antibody) test results were included. The gold standard for SLE diagnosis was expert opinion. Results The sensitivities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 68.7%, 95.4%, and 91.6%, respectively. The specificities of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 criteria were 94.8%, 89.7%, and 88.5%, respectively. 18 SLE patients met the SLICC 2012 but not the EULAR/ACR 2019 criteria. Among these, hematologic involvement was prominent (13/18; 72.2%). Eight SLE patients fulfilled the EULAR/ACR 2019 but not the SLICC 2012 criteria. Among these, joint involvement was prominent (6/8; 75%). Conclusion This is the largest cohort study of pediatric SLE testing the performances of all three classification criteria. The SLICC 2012 criteria yielded the best sensitivity, while the ACR 1997 criteria had the best specificity. SLICC 2012 criteria performed better than EULAR/ACR 2019 criteria. Separation of different hematological manifestations in the SLICC 2012 criteria might have contributed to the higher performance of this criteria set.

Sign in / Sign up

Export Citation Format

Share Document