Study of Antiphospholipid Antibodies in Patients with Arterial Hypertension

Antiphospholipid syndrome (APS) is a multifactorial, autoantibody-mediated disease. Antiphospholipid antibodies (aPL) directed against negatively charged phospholipids or various combinations of phospholipid-binding proteins seem to be an independent pathogenic factor that plays a critical role in APS. Unfortunately, their role in hypertension is not fully elucidated. The aim of our study was to determine aPL titers in hypertension patients and investigate the association of aPL with renal impairment parameters. Forty-seven patients with arterial hypertension (22 males, 46.8% and 25 females, 53.2%), aged 41–85 years old (mean 65.9 ± 10.1 years), and 21 age-sex-matched subjects without severe hypertension as control group (8 males, 13 females, 38.1% vs. 61.9%), mean age 61 ± 11.3 years, were enrolled in this study. Patients with other risk factors like Rheumatoid Arthritis and Systematic Lupus Erythematosus (SLE), both viral and bacterial acute infections, and cancer were excluded from the study. The aPL (anticardiolipin (ACA) and anti-b2GPI antibodies, IgG and IgM) were measured by ELISA (Aesculisa, Aesku Diagnostics, Wendelsheim, Germany) with a cutoff of 15 GPL/MPL for ACA and 15 U/mL for b2GPI. Serum Neutrophil gelatinase-associated lipocalin (sNGAL) was measured by ELISA kits (BioVendor, Brno, Czech Republic). Biochemical analysis such as serum creatinine (Cr), were measured by automated analyzer and finally estimated glomerular filtration rate (e-GFR) was calculated by the Chronic Kidney Disease Epidemiology Collaboration (CKD-EPI). Fifteen patients were positive for ACA IgG (31.9%), two for anti-b2GPI IgM (4.2%), and three for anti-b2GPI IgG (6.3%). Furthermore, three persons from control group were positive in anti-b2GPI IgG (14.27%). The serum level of anti-b2GPI IgG was significantly higher in patients compared to healthy controls (p = 0.013). The level of sNGAL (59.63 ± 41.5 ng/mL vs. 45.5 ± 21.5 ng/mL, p = 0.14) was not higher in hypertensive patients than in the age-sex-matched control group. Additionally, the sNGAL level was found to be directly and positively correlated in patients with positive ACA IgG (r2 = 0,945, p < 0.0001). These results demonstrate that autoimmunity may be one of the pathogenetic factors of hypertension and aPL antibodies might be a potential marker of renal involvement.

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AB0007 STAT4 RS7574865 G/T POLYMORPHISM AS MARKER OF PREDISPOSITION TO SYSTEMIC LUPUS ERYTHEMATOSUS WITH JUVENILE ONSET

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.2142 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1038.2-1039

Author(s):

M. Kaleda ◽

M. Krylov ◽

I. Nikishina

Keyword(s):

Systemic Lupus Erythematosus ◽

Pilot Study ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Control Group ◽

Coombs Test ◽

Systemic Lupus ◽

Direct Coombs Test ◽

Positive Direct ◽

Cutaneous Lupus

Background:Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a significant genetic predisposition. Recent studies have identified STAT4 (signal transducers and transcription activators 4) as a susceptibility gene for SLE.Objectives:To investigate the hypothesis of the association of STAT4 rs7574865 G/T polymorphism with the predisposition to SLE in children and its relationship with some of SLE manifestations.Methods:The case-control pilot study included 143 children (39 with SLE and 103 healthy unrelated volunteers as a control group). Diagnosis of SLE was based on 2012 SLICC criteria. STAT4 rs7574865 G/T polymorphism was investigated using allele-specific real-time polymerase chain reaction (RT-PCR).Results:The group of pts with SLE consisted of 29 girls and 10 boys, with an average age of 11.8±3.7 years (from 3 to 17 years) and an average disease duration of 4.1±2.4 years. 79.5% pts had acute cutaneous lupus at the onset, 46.1% - nonscarring alopecia, 71.8% - arthritis, 23.1% - oral and nasal ulcers, 23.1% - serositis, 43.6% - renal involvement, 35.9% –neuropsychiatric disorders. Leucopenia/lymphopenia was found in 71.8% of pts, thrombocytopenia – in 23,1%. ANA were detected in 100% pts, anti-dsDNA – in 79.5%, anti-Sm – in 31.6%, antiphospholipid antibodies - in 7,3%, hypocomplementemia – in 61.5%, positive direct Coombs test – in 35.9 %. Macrophage activation syndrome at the onset was documented in 15.4 % of pts. The distribution of rs7574865 genotypes in the control group showed no significant deviations from the Hardy-Weinberg equilibrium. The distribution of genotype frequencies among pts had statistically significant differences compared to the control (χ2=12.95, p=0.0015): GG-30.8% and 63.1% (p=0.001), GT-56.4% and 33.0% (p=0.018), TT-12.8% and 3.9% (p=0.114), GT+TT - 69.2% and 36.9% (p=0.0005). The frequency of the mutant STAT 4 allele T (polymorphism), was significantly higher in the SLE group than in the control group (41% and 20.4%, respectively; p=0.0007). We identified an association of the T allele with some clinical, laboratory, and immunological disorders in SLE: arthritis (OR 3.9, p=0.0002), acute cutaneous lupus (OR 2.47, p=0.003), nonscarring alopecia (OR 3.12, p=0.002), renal involvement (OR 2.42, p=0.022), leucopenia (OR 2.72, p=0.003), thrombocytopenia (OR 4.88, p=0.002), anti-dsDNA (OR 2.82, p=0.0006), hypocomplementemia (OR 2.34, p=0.012), positive direct Coombs test (OR 3.38, p=0.002).Conclusion:Our pilot study confirmed that the STAT4 rs7574865 G/T polymorphism was associated with the risk of SLE in children and some of SLE manifestations.Disclosure of Interests:None declared

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AB0423 NEUROPSYCHIATRIC OUTCOME OF CHILDREN BORN TO WOMEN WITH SYSTEMIC LUPUS ERYTHEMATOSUS (SLE) WOMEN AND EXPOSED IN UTERO TO AZATHIOPRINE: A CASE-CONTROL STUDY.

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4912 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1511.2-1511

Author(s):

M. G. Lazzaroni ◽

F. Crisafulli ◽

I. Debeni ◽

C. Nalli ◽

L. Andreoli ◽

...

Keyword(s):

Lupus Erythematosus ◽

Renal Involvement ◽

In Utero ◽

Learning Disorders ◽

Control Group ◽

In Utero Exposure ◽

Term Outcome ◽

Similar Frequency ◽

Long Term Outcome

Background:A possible increase in neurodevelopmental (ND) and learning disorders (LD) in the offspring of mothers affected by SLE have been suggested in some studies, along with the identification of different possible risk factors. Azathioprine (AZA) is commonly used during pregnancy, based on its non-teratogenicity and extended experience in women with different diseases. However, a few small studies suggested an association between in utero exposure to AZA and possible increased frequency of ND/LD in children, indirectly derived from increased request of supportive educational services.Objectives:To evaluate the medium-long term outcome in terms of ND/LD in children of school age (≥6 years) born to SLE women treated with AZA during pregnancy, as compared to that of children born to SLE mothers not treated with AZA during pregnancy.Methods:Data from our Pregnancy Clinic registry were collected for prospectively followed pregnancies of SLE women treated with AZA (cases) and compared to pregnancies of SLE women not treated with AZA (controls), that were matched for age at pregnancy, presence of renal involvement and aPL positivity. SLE patients (cases and controls) were interviewed by phone to collect data about their children, focusing on the presence of ND/LD certified by Neuropsychiatrists.Results:Data were collected for 14 SLE mothers in the AZA group and 31 in the control group, with similar age at pregnancy (30.3±5.21 vs 31.4±4.70 years, p:0.45) and frequency of renal involvement (50.0% vs 44.1%, p:0.77), aPL positivity (33.3% vs 29.4%, p:0.76) and anti-Ro/SSA positivity (27.8% vs. 26.5%, p:0.55). A SLE flare during pregnancy was more frequently recorded in the AZA group (27.8% vs. 2.94%, p:0.02). Other medications included HCQ (55.6% vs. 70.6%, p:0.36) and corticosteroids (100% vs 79.4%, p:0.08).We collected data for 18 children in the AZA group and 34 children in the control group, that had a similar mean age at the time of the interview (12.7±4.80 vs. 12.9±5.61 years, p:0.91). The two groups had also similar gestational age (37.4±2.20 weeks vs. 38.0±1.29 weeks, p:0.23), birth weight (3003±433 g vs 3011±453 g, p:0.95) and rate of male sex (61.1% vs 44.1%, p:0.38).We recorded similar frequency of ND/LD in the two groups. In particular, a ND was present in 2/18 (11.1%) of children exposed to AZA vs. 2/34 (5.88%) in the control group (p:0.60). A LD was present in 1/18 cases (5.56%) and 6/34 controls (17.6%) (p:0.40).Conclusion:The medium-long term outcome of children born to SLE mothers in the whole cohort was characterized by the presence of ND in 4/54 (7.69%) and LD in 7/52 (13.5%). ND/LD do not seem to be related to in utero exposure to AZA.Disclosure of Interests:None declared

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Serum and urine TNF-like weak inducer of apoptosis, monocyte chemoattractant protein-1 and neutrophil gelatinase-associated lipocalin as biomarkers of disease activity in patients with systemic lupus erythematosus

Lupus ◽

10.1177/0961203320904997 ◽

2020 ◽

Vol 29 (4) ◽

pp. 379-388 ◽

Cited By ~ 2

Author(s):

S Mirioglu ◽

S Cinar ◽

H Yazici ◽

Y Ozluk ◽

I Kilicaslan ◽

...

Keyword(s):

Disease Activity ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Systemic Lupus ◽

Monocyte Chemoattractant Protein 1 ◽

Monocyte Chemoattractant ◽

Neutrophil Gelatinase Associated Lipocalin ◽

Neutrophil Gelatinase ◽

Urine Levels ◽

Serum And Urine

Objectives TNF-like weak inducer of apoptosis (TWEAK), monocyte chemoattractant protein-1 (MCP-1) and neutrophil gelatinase-associated lipocalin (NGAL) are proinflammatory cytokines/chemokines that are considered as potential biomarkers reflecting disease activity in systemic lupus erythematosus (SLE). In this study, we aimed to investigate the association of serum (s) and urine (u) levels of TWEAK, MCP-1 and NGAL with disease activity in both renal and extra-renal SLE. Methods Thirty active patients with SLE (15 renal and 15 extra-renal) were recruited. Thirty-one inactive patients with SLE (16 renal and 15 extra-renal), 14 patients with ANCA-associated vasculitis (AAV) all of whom had active renal involvement and 20 healthy volunteers were selected as control groups. Serum and urine levels of TWEAK, MCP-1 and NGAL were tested using ELISA. Results Serum and urine levels of TWEAK and NGAL were significantly higher in the active SLE group compared to the inactive SLE group (sTWEAK p = 0.005; uTWEAK p = 0.026; sNGAL p < 0.001; uNGAL p = 0.002), whilst no significant differences regarding serum and urine MCP-1 levels were observed ( p = 0.189 and p = 0.106, respectively). uTWEAK ( p = 0.237), sMCP-1 ( p = 0.141), uMCP-1 ( p = 0.206), sNGAL ( p = 0.419) and uNGAL ( p = 0.443) levels did not differ between patients with active renal and extra-renal SLE. Serum TWEAK was higher in patients with active renal SLE ( p = 0.006). There were no differences between active renal SLE and active renal AAV. Levels of all biomarkers were correlated with the SLE Disease Activity Index. Conclusion sTWEAK, uTWEAK, sNGAL and uNGAL are biomarkers showing disease activity in SLE. However, our results implicate that these biomarkers may not be specific for SLE, and can be elevated in patients with active renal involvement of AAV.

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Urine peptidomic biomarkers for diagnosis of patients with systematic lupus erythematosus

Lupus ◽

10.1177/0961203317707827 ◽

2017 ◽

Vol 27 (1) ◽

pp. 6-16 ◽

Cited By ~ 11

Author(s):

M Pejchinovski ◽

J Siwy ◽

W Mullen ◽

H Mischak ◽

M A Petri ◽

...

Keyword(s):

Early Diagnosis ◽

Lupus Erythematosus ◽

Biomarker Discovery ◽

Renal Involvement ◽

Disease Onset ◽

Organ Damage ◽

Amino Acid Sequences ◽

Systematic Lupus Erythematosus ◽

Non Invasive ◽

Improve Patient

Background Systematic lupus erythematosus (SLE) is characterized with various complications which can cause serious organ damage in the human body. Despite the significant improvements in disease management of SLE patients, the non-invasive diagnosis is entirely missing. In this study, we used urinary peptidomic biomarkers for early diagnosis of disease onset to improve patient risk stratification, vital for effective drug treatment. Methods Urine samples from patients with SLE, lupus nephritis (LN) and healthy controls (HCs) were analyzed using capillary electrophoresis coupled to mass spectrometry (CE-MS) for state-of-the-art biomarker discovery. Results A biomarker panel made up of 65 urinary peptides was developed that accurately discriminated SLE without renal involvement from HC patients. The performance of the SLE-specific panel was validated in a multicentric independent cohort consisting of patients without SLE but with different renal disease and LN. This resulted in an area under the receiver operating characteristic (ROC) curve (AUC) of 0.80 ( p < 0.0001, 95% confidence interval (CI) 0.65–0.90) corresponding to a sensitivity and a specificity of 83% and 73%, respectively. Based on the end terminal amino acid sequences of the biomarker peptides, an in silico methodology was used to identify the proteases that were up or down-regulated. This identified matrix metalloproteinases (MMPs) as being mainly responsible for the peptides fragmentation. Conclusions A laboratory-based urine test was successfully established for early diagnosis of SLE patients. Our approach determined the activity of several proteases and provided novel molecular information that could potentially influence treatment efficacy.

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Comparative analysis of neutrophil gelatinase-associated lipocalin and other laboratory markers for lupus nephritis: a cross-sectional investigation

Annals of Clinical Biochemistry International Journal of Laboratory Medicine ◽

10.1177/0004563216651888 ◽

2016 ◽

Vol 54 (2) ◽

pp. 240-245 ◽

Cited By ~ 2

Author(s):

Sonia L La’ulu ◽

Brenda B Suh-Lailam ◽

K Wayne Davis ◽

Joely A Straseski ◽

Anne E Tebo

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Nephritis ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Immunofluorescence Assay ◽

Igg Antibodies ◽

Systemic Lupus ◽

Neutrophil Gelatinase Associated Lipocalin ◽

Double Stranded Dna ◽

Neutrophil Gelatinase

Background Lupus nephritis is one of the most serious complications of systemic lupus erythematosus. This study evaluates the prevalence and correlation between neutrophil gelatinase-associated lipocalin and other biomarkers associated with renal involvement in systemic lupus erythematosus. Methods Paired serum and urine specimens from 50 suspected systemic lupus erythematosus patients, characterized by antinuclear antibodies detected by indirect immunofluorescence assay and varying positive concentrations of anti-double stranded DNA antibodies by Crithidia luciliae immunofluorescence assay, were investigated. Of these 50 patients, 18 were identified with renal involvement based upon laboratory serology. Patients and healthy control serum samples ( n = 50) were also evaluated for high avidity double stranded DNA IgG antibodies, anti-C1q IgG antibodies, and serum creatinine. The prevalence and relationship between biomarkers were evaluated using statistical methods. Results Serum and urine neutrophil gelatinase-associated lipocalin concentrations were significantly elevated in patients compared to controls, with a prevalence of 24% and 36%, respectively. These concentrations were also more markedly increased in systemic lupus erythematosus patients with renal involvement than those without. Spearman’s correlations between neutrophil gelatinase-associated lipocalin and other biomarkers tested ranged from 0.06 to 0.66 in all patients. Combined concordance as determined by Cronbach alpha coefficient between biomarkers was reduced from 0.71 to 0.58 (serum) and 0.62 (urine) when neutrophil gelatinase-associated lipocalin was removed. Conclusions Neutrophil gelatinase-associated lipocalin concentrations are elevated and demonstrate variable associated with other laboratory markers for renal involvement in systemic lupus erythematosus. Prospective longitudinal studies are needed to determine the optimal biomarker combinations for use in routine management of systemic lupus erythematosus patients at-risk for lupus nephritis.

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Serum markers thrombophilia in pregnant women with Systemic Lupus Erythematosus

Revista Brasileira de Saúde Materno Infantil ◽

10.1590/1806-93042017000400012 ◽

2017 ◽

Vol 17 (4) ◽

pp. 833-842

Author(s):

Vanessa Marcon de Oliveira ◽

Ernesto Antonio Figueiró-Filho ◽

Cristiane Munaretto Ferreira ◽

Erica Freire de Vasconcelos Pereira

Keyword(s):

Systemic Lupus Erythematosus ◽

Pregnant Women ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Serum Markers ◽

Control Group ◽

Systemic Lupus ◽

Exact Test ◽

Hereditary Thrombophilia ◽

Acquired Thrombophilia

Abstract Objectives: to determine the frequency of serum markers for hereditary and acquired thrombophilia and their association with pregnancy in women with Systemic Lupus Erythematosus (SLE). Methods: a case-control study was conducted among 25 pregnant women with SLE (study group) and 32 pregnant women without known disease and with at least one previous pregnancy (control group). The presence of antiphospholipid antibodies and hereditary thrombophilia were examined in both groups. We used the y2 Test with Yates correction or Fisher's Exact Test to verify the associations and calculate the relative risk. Results: thrombophilia was present in 72.0% of pregnant women with SLE and in 6.0% of patients in the control group. A significant association was found between the presence of SLE and serum markers for hereditary thrombophilia / antiphospholipid antibodies (p<0.05). The relative risks for antiphospholipid antibodies were 13.20 (ICR95%= 1.81 - 96.46) in pregnant women with SLE, 7.26 (CI95%= 1.77 - 29.86) for the presence of serum markers of hereditary thrombophilia and 7.92 (CI95%= 2.62 - 3.94) for the presence of hereditary thrombophilia and/or antiphospholipid antibodies. Conclusions: the identification of markers for hereditary and/or acquired thrombophilia in pregnant women with lupus may be clinically useful to determine which patients have a higher risk of obstetric complications.

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AB0314 SEMAPHORIN 3A LEVELS IN LUPUS WITH AND WITHOUT SECONDARY ANTIPHOSPHOLIPID ANTIBODY SYNDROME AND RENAL INVOLVEMENT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.1864 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 1182.2-1182

Author(s):

G. S. Kart-Bayram ◽

D. Bayram ◽

A. Erden ◽

S. C. Güven ◽

B. Özdemir ◽

...

Keyword(s):

Lupus Erythematosus ◽

Renal Involvement ◽

Control Group ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Single Group ◽

Group A ◽

Patient Groups ◽

Group B ◽

And Control

Background:In this study, we aimed to evaluate sema3A levels in SLE patients with and without renal involvementor secondary antiphospholipid antibody syndrome (APS), to further elucidate the contribution ofsema3A in etiopathogenesis these conditionsObjectives:Aim of this study is to evaluate sema3A levels in systemic lupus erythematosus patients (SLE) with and without renal involvement and secondary antiphospholipid antibody syndrome (APS).Methods:SLE patients were grouped according to presence of secondary APS or renal involvement. The control group consisted of age-matched, non-smoker, healthy volunteers. Sema3A levels were compared among groups. All SLE patients were regrouped according to presence of thrombotic events, miscarriages and proteinuria and sema3A levels were investigated. Finally, sema3A levels of all SLE patients as a single group were compared to controls.Results:The mean sema3A values were 16.16±2.84 ng/dL in the control group, 11.28±5.23 ng/dL in SLE patients without nephritis and APS, 9.05±5.65 ng/dL in SLE with APS group, and 8.53±5.11 ng/dL in lupus nephritis group. When all three patient groups were examined as a single group, mean sema3A value was significantly lower than that of the control group. Sema3A was reduced in SLE patients with thromboembolism and/or miscarriage.Conclusion:Sema3A levels were lower in all patient groups compared to the control group. Moreover, the reduced sema3A levels in patients with a history of thromboembolism and/or miscarriage suggests that sema3A may play an important role in the pathogenesis of vasculopathyTable 1.Comparison of sema3A levels between SLE patient groups and control subjectsPatient groupspGroup A (N=20)Group B (N=20)Group C (N=19)Control (N=19)Sema3A, ng/dL, mean ± SD9.05 ± 5.6511.28 ± 5.238.53 ± 5.1116.16 ± 2.84Group A vscontrol<0.001Group B vscontrol<0.001Group C vscontrol<0.001Group A vs B = 0.203Group A vs C = 0.766Group B vs C = 0.106<0.001All patients (N=59)Control (N=19)9.64 ± 5.3816.16 ± 2.84Patients with thrombotic events and/or miscarriages (N=31)Patients without thrombotic events and/or miscarriages (N=48)0.0329.96 ± 5.1112.33 ± 5.84<0.001Patients with proteinuria and/or thrombotic events and/or miscarriages (N=45)Patients without proteinuria and/or thrombotic events and/or miscarriages (N=34)9.05 ± 5.0914.91± 4.50Disclosure of Interests:None declared

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Low Urine Secretion of Semaphorin3A In Lupus Patients With Proteinuria

10.21203/rs.3.rs-750580/v1 ◽

2021 ◽

Author(s):

Doron Rimar ◽

Merav Lidar ◽

Nasrin Eiza ◽

Adi D Sabag ◽

Elias Toubi ◽

...

Keyword(s):

Immune Responses ◽

Disease Activity ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Control Group ◽

Healthy Controls ◽

Adaptive Immune ◽

Urine Concentrations ◽

Urine Levels

Abstract Background: Immune semaphorins are important players in controlling both innate and adaptive immune responses. The regulatory role of semaphorin3A (sema3A) in systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and other autoimmune diseases is widely reported. Decreased levels of serum sema3A were shown to be associated with SLE disease activity. Objectives: To assess urine concentrations of sema3A in SLE patients and its correlation with renal involvement and disease activity. Methods: Urine levels of sema3A were analyzed in 38 SLE patients of whom 13 had renal involvement and were compared to 10 healthy controls and 8 RA patients (disease control group). Results: The secretion of urine sema3A was found to be significantly lower in SLE patients compared to healthy controls and RA patients (4.9±3.9 ng/ml, 8.5±2.7 ng/ml, 9.85±1.7 ng/ml, respectively, p = 0.0006). Urine sema3A was significantly lower in SLE patients with lupus nephritis than in patients without nephritis (4.0±3.4 ng/ml vs 6.5±3.8 ng/ml, p=0.03). Urine sema3A was inversely correlated with proteinuria and SLE disease activity. Conclusion: Urine sema3A is decreased in lupus patients and should be further evaluated as a possible biomarker for disease activity and renal involvement.

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

Lupus ◽

10.1177/09612033211038824 ◽

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.

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