Clinical Manifestation and Hematologic Interpretation of Pediatric Systematic Lupus Erythematosus at Initial Presentation:  2-Years Observation

Background: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by a spectrum of clinical manifestations, immunological abnormalities, and varied laboratories results. In children, SLE manifestation is particularly more severe, involving more organs. Hematological manifestation has been known as the most common manifestation. The purpose of this study was to describe the clinical manifestations and hematologic interpretation of pediatric SLE at initial presentation.Methods: This retrospective data collection study was conducted at the Department of Child Health Dr. Hasan Sadikin General Hospital Bandung on medical records from a two-year period of 2017–2018. The clinical manifestations were categorized into malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, renal disorders, and neurological disorders. The hematologic interpretations were categorized into anemia, leukopenia, neutropenia, lymphopenia, and thrombocytopenia. Clinical manifestations and hematological interpretations were presented as occurrence percentages and stratified into three age-group of pre-pubertal, peri-pubertal, and post-pubertal.Results: Among 79 pediatric SLE patients (median age 14 years old; IQR 11–16), female gender was predominant. Abnormalities hematologic interpretation occurs in more than half of the patients (83.5%). Malar rash and anemia were the commonest findings among all age groups. Increased occurrence of neuropsychiatric and renal disorders were observed in all age-groups.Conclusions: Malar rash and anemia are important findings among pediatric SLE patients. Furthermore, the occurrences of the neuropsychiatric and renal disorders are also important.

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SAT0217 PERFORMANCE OF ACR/EULAR 2019, SLICC 2012 AND ACR 1997 CLASSIFICATION CRITERIA IN A COHORT OF SYSTEMIC LUPUS ERYTHEMATOSUS PATIENTS WITH LONGSTANDING DISEASE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6190 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1051-1052

Author(s):

D. Lobo Prat ◽

B. Magallares ◽

I. Castellví ◽

H. Park ◽

P. Moya ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Statistical Significance ◽

Renal Involvement ◽

High Sensitivity ◽

Classification Criteria ◽

Average Score ◽

Systemic Lupus ◽

Malar Rash ◽

Oral Ulcers

Background:Systemic lupus erythematosus (SLE) is an autoimmune disease with variable clinical features and a complex physiopathology. In 2019, EULAR and ACR have jointly developed new classification criteria with both high sensitivity and specificity. These criteria have the particularity of including the presence of ANA as an obligatory entry criterion and the existence of clinical and immunological domains with weighted scores.Objectives:To evaluate the performance and characteristics of the ACR/EULAR 2019, SLICC 2012 and ACR 1997 classification criteria in a cohort of SLE patients with longstanding disease.Methods:Descriptive observational study that enrolled a cohort of SLE patients with longstanding disease followed in a tertiary level hospital. Demographic and clinical data were gathered along with the fulfillment of classification criteria. The sensitivity of each classification criteria and the statistically significant associations between criteria fulfillment and clinical and immunological data were calculated. Statistical analyses were performed using the Chi2, T-student and ANOVA tests. Statistical significance was assumed in p values <0.05.Results:A total of 79 patients (88.6% women) with a mean age of 51.8±14 years, disease duration of 15.2±11.5 years and SLEDAI of 2.65±2.1 were included. The sensitivity of the different classification criteria was 51.9% for ACR 1997, 87.3% for SLICC 2012 and 86.1% for ACR/EULAR 2019 (Table 1).Table 1.Sensitivity and average scores.ACR/EULAR 2019SLICC 2012ACR 1997Sensitivity (%)86.187.351.9Average score of patients classified as SLE(±SD)18.6±5.85.3±1.45±0.9Average score of patients NOT classified as SLE(±SD)6.1±2.52.8±0.42.8±0.851.9% of patients met all three classification criteria, 29.1% met SLICC 2012 and ACR/EULAR 2019, 5% only met SLICC 2012 and 3.7% exclusively met ACR/EULAR 2019. 11.4% of patients did not meet any classification criteria and were characterized by having a low SLEDAI (0.6±0.9) and fulfilling only skin domains (alopecia or oral ulcers), antiphospholipid antibodies or hypocomplementemia.Statistically significant associations were found between meeting ACR/EULAR 2019 classification criteria and the presence of low C3 and C4 (p<0.04), DNA (p<0.001), lupus nephritis III-IV (p<0.05) and arthritis (p<0.001), highlighting that all patients with arthritis met these criteria.In the SLICC 2012 evaluation, significant associations were found between meeting these criteria and the presence of arthritis (p<0.01), renal involvement (p<0.04), leukopenia/lymphopenia (p=0.05), DNA (p<0.03) and hypocomplementemia (p=0.02).Fullfilment of ACR 1997 was associated to the presence of malar rash (p<0.001), discoid lupus (p<0.05), photosensitivity (p<0.001) and oral ulcers (p<0.04), as well as arthritis (p<0.001), serositis (p=0.02), renal (p<0.05) and hematologic (p=0.05) involvement.The Kappa concordance coefficient among classification criteria is detailed in Table 2.Table 2.Kappa concordance coefficient.ACR/EULAR 2019 - SLICC 2012ACR/EULAR 2019 - ACR 1997SLICC 2012 - ACR 1997Kappa concordance coefficient0.610.270.30Conclusion:The ACR/EULAR 2019 classification criteria maintain a high sensitivity similar to the SLICC 2012 in SLE patients with longstanding disease, both of which are much higher than ACR 1997. Patients with serological, articular or renal involvement are more likely to meet SLICC 2012 or ACR/EULAR 2019 criteria. It is noteworthy the relevance of dermatological manifestations in ACR1997 classification criteria against the increased weight that a better understanding of SLE physiopathology has provided to analytic and immunological criteria in the subsequent classification criteria.Disclosure of Interests:David Lobo Prat: None declared, Berta Magallares: None declared, Ivan Castellví Consultant of: Boehringer Ingelheim, Actelion, Kern Pharma, Speakers bureau: Boehringer Ingelheim, Actelion, Bristol-Myers Squibb, Roche, HyeSang Park: None declared, Patricia Moya: None declared, Ignasi Gich: None declared, Ana Laiz: None declared, Cesar Díaz-Torné: None declared, Ana Milena Millán Arciniegas: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared

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The lupus patient with positive rheumatoid factor

Lupus ◽

10.1177/0961203318759607 ◽

2018 ◽

Vol 27 (8) ◽

pp. 1368-1373 ◽

Cited By ~ 5

Author(s):

A Fedrigo ◽

T A F G dos Santos ◽

R Nisihara ◽

T Skare

Keyword(s):

Rheumatoid Factor ◽

Lupus Erythematosus ◽

Univariate Analysis ◽

Disease Onset ◽

Clinical Manifestations ◽

Epidemiological Data ◽

Positive Rheumatoid Factor ◽

Serological Profile ◽

Oral Ulcers ◽

Rheumatology Unit

Background Patients with systemic lupus erythematosus (SLE) may form clusters with clinical manifestations and autoantibodies. Objective The objective of this report is to study whether SLE patients with positive rheumatoid factor (RF) have a special clinical and/or serological profile. Methods A retrospective study of 467 SLE patients seen at a single rheumatology unit was conducted. Epidemiological data (age, gender, age at disease onset, ethnic background and tobacco use), clinical data (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia, arthritis and hypothyroidism) and serological profile (anti-dsDNA, anti-Ro/SS-A, anti-La/SS-B, anti-RNP, anti-Sm, IgG aCL, IgM aCL, lupus anticoagulant, direct Coombs and RF) were collected. Patients with positive and negative RF were compared. Results RF was found in 24.9% of the sample. In univariate analysis, RF was positively associated with butterfly rash ( p = 0.04), anti-Ro ( p = 0.03), anti-Sm antibodies ( p = 0.01) and hypothyroidism ( p = 0.01) and negatively associated with glomerulonephritis ( p = 0.003). Logistic regression showed that only glomerulonephritis ( p = 0.03; OR = 0.45; 95% CI = 0.21–0.93) and anti-Ro ( p = 0.009; OR = 2.3; 95% CI = 1.24–4.57) were independent associations. Conclusion In our sample RF was associated with protection from glomerulonephritis and with higher prevalence of anti-Ro antibodies.

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Predictors of Incident Seizure in Systemic Lupus Erythematosus

The Journal of Rheumatology ◽

10.3899/jrheum.150135 ◽

2016 ◽

Vol 43 (3) ◽

pp. 565-575 ◽

Cited By ~ 12

Author(s):

XiangYang Huang ◽

Laurence S. Magder ◽

Michelle Petri

Keyword(s):

Risk Factors ◽

Systemic Lupus Erythematosus ◽

Disease Activity ◽

Lupus Erythematosus ◽

Renal Involvement ◽

Multivariable Analysis ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Malar Rash ◽

History Of

Objective.The risk factors for incident seizures in systemic lupus erythematosus (SLE) were prospectively determined in a cohort study.Methods.A total of 2203 patients with SLE followed longitudinally in the Hopkins Lupus Cohort were analyzed. Demographic variables, clinical manifestations, laboratory tests, and SLE disease activity were recorded at each quarterly visit. Adjusted estimates of association of risk factors for onset of seizure were derived using pooled logistic regression. We examined incident seizures in 3 ways: at the time of diagnosis, more than 45 days after the diagnosis of SLE, and after cohort entry.Results.Of 2203 patients with no history of seizure prior to SLE diagnosis, 157 (7.13%) had the first seizure occurrence at the time of (37 patients, 1.68%) or after diagnosis (120 patients, 5.45%) of SLE. The risk of seizure occurring around the time of SLE diagnosis was higher in patients with a history of malar rash (p = 0.002), proteinuria (p = 0.004), and psychosis (p < 0.001). Multivariable analysis of the first seizure occurring after the diagnosis of SLE showed that history of low C3 (p = 0.0078), psychosis (p < 0.0001), cranial or peripheral neuropathy (p = 0.0043), anti-Sm antibody (p = 0.0551), renal involvement (p = 0.0177), and current corticosteroid dose (p < 0.0001) were independently associated with a higher incidence of seizure. Disease activity was not predictive after adjusting for corticosteroids.Conclusion.Risk of seizure after diagnosis of SLE is increased in those patients with prior psychosis, neuropathy, proteinuria, anti-Sm, low C3, and use of corticosteroids.

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Potential for Antigen-Specific Tolerizing Immunotherapy in Systematic Lupus Erythematosus

Frontiers in Immunology ◽

10.3389/fimmu.2021.654701 ◽

2021 ◽

Vol 12 ◽

Author(s):

Sean Robinson ◽

Ranjeny Thomas

Keyword(s):

Lupus Erythematosus ◽

Survival Rates ◽

Clinical Manifestations ◽

Treg Cells ◽

Immunological Tolerance ◽

Immunosuppressive Drugs ◽

Systemic Autoimmune Disease ◽

Systematic Lupus Erythematosus ◽

Curative Therapy

Systemic lupus erythematosus (SLE) is a chronic complex systemic autoimmune disease characterized by multiple autoantibodies and clinical manifestations, with the potential to affect nearly every organ. SLE treatments, including corticosteroids and immunosuppressive drugs, have greatly increased survival rates, but there is no curative therapy and SLE management is limited by drug complications and toxicities. There is an obvious clinical need for safe, effective SLE treatments. A promising treatment avenue is to restore immunological tolerance to reduce inflammatory clinical manifestations of SLE. Indeed, recent clinical trials of low-dose IL-2 supplementation in SLE patients showed that in vivo expansion of regulatory T cells (Treg cells) is associated with dramatic but transient improvement in SLE disease markers and clinical manifestations. However, the Treg cells that expanded were short-lived and unstable. Alternatively, antigen-specific tolerance (ASIT) approaches that establish long-lived immunological tolerance could be deployed in the context of SLE. In this review, we discuss the potential benefits and challenges of nanoparticle ASIT approaches to induce prolonged immunological tolerance in SLE.

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Circulating levels of hypoxia-inducible factor-1α and miR-210 are associated with photosensitivity in systemic lupus erythematosus patients

Current Molecular Medicine ◽

10.2174/1566524022666220114145220 ◽

2022 ◽

Vol 22 ◽

Author(s):

Amira M. Gamal-Eldeen ◽

Cinderella A. Fahmy ◽

Bassem M. Raafat ◽

Fayez Althobaiti ◽

Iman H. Bassyouni ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Activity Index ◽

White Blood Cells ◽

Hypoxia Inducible Factor ◽

Disease Activity Index ◽

Strong Positive Correlation ◽

Systemic Lupus ◽

Renal Disorders ◽

Oral Ulcers

Background: miR-210, a key HypoxamiR, regulates the hypoxia and inflammation-linked hypoxia. Systemic lupus erythematosus (SLE), a chronic autoimmune disease, responsible for many pathological disorders, including photosensitivity. Objective: Finding the correlation between the circulating miR-210/HIF-1α levels and photosensitivity in SLE patients and other SLE-associated pathological complications, in a single-center case control study. Methods: Study population of 104 SLE Egyptian patients with photosensitivity, 32 SLE patients without photosensitivity, and 32 healthy subjects. SLE activity was assessed for all patients by SLE Disease Activity Index (SLEDAI). The clinical complications/manifestations and the hematological/serological analyses were recorded. HIF-α concentration was investigated by ELISA and miR-210 expression was analyzed by qRT-PCR. Results: The results revealed that circulating miR-210 was significantly increased in SLE/photosensitivity than SLE and controls. The additional occurrence of malar rash, oral ulcers, renal disorders or hypertension resulted in a higher expression of miR-210. SLEDAI activity status showed no effect on miR-210. Erythrocyte sedimentation rate, white blood cells, hemoglobin, platelets, the patients age and the disease duration were positively correlated with circulatory miR-210. HIF-α concentration was significantly induced in SLE/photosensitivity than SLE and controls. In SLE/photosensitivity, presence of renal disorders and hypertension resulted in highest HIF-α concentrations. A strong positive correlation was recorded between HIF-α concentration and circulatory miR-210 in SLE/photosensitivity patients (r = 0.886). Conclusion:: The dysregulation of circulating miR-210/ HIF-1α levels in SLE/photosensitivity‎ patients is controlled by the presence of additional pathological complications and supposed that hypoxia pathway might interact positively with the pathogenesis and illness progress of SLE.

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A Self-Limited Facial Rash in a Lupus Patient: The Case of Primary Facial Raynaud’s Phenomenon

Case Reports in Dermatology ◽

10.1159/000517553 ◽

2021 ◽

pp. 366-371

Author(s):

Kamel El-Reshaid ◽

Shaikha Al-Bader ◽

Hossameldin Tawfik Sallam

Keyword(s):

Systemic Lupus Erythematosus ◽

Case Report ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Raynaud’S Phenomenon ◽

Raynaud's Phenomenon ◽

Joint Disease ◽

Systemic Lupus ◽

Malar Rash ◽

Oral Ulcers

Skin is involved in 80% of systemic lupus erythematosus (SLE) and the second most affected after joint disease. Lupus-specific lesions include (a) acute ones viz. malar rash (80%), (b) subacute ones viz. photosensitive maculopapular dermatitis (50%), and (c) chronic ones viz. discoid rash. The lupus nonspecific lesions include; (a) nonscarring alopecia (86.67%), oral ulcers (56.67%), vasculitic lesions (33.34%), bullous lesions (10%), and Raynaud’s phenomenon (6.67%). In this case report, we describe a patient with SLE and antiphospholipid antibodies that had developed a transient facial form of Raynaud’s phenomenon that was not associated with disease activity and digital changes. Its association with SLE is discussed.

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Clinical and immunological pattern and outcome of Egyptian systemic lupus erythematosus patients: a single center experience

Lupus ◽

10.1177/0961203318776085 ◽

2018 ◽

Vol 27 (9) ◽

pp. 1562-1569 ◽

Cited By ~ 9

Author(s):

G A Mahmoud ◽

A A Shahin ◽

H S Zayed ◽

A Moghazy ◽

B M Eissa

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Survival Rates ◽

Disease Onset ◽

Elevated Serum ◽

Damage Index ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Oral Ulcers ◽

Increased Risk

Objective The objective of this study was to describe the clinical and immunological pattern and disease outcome in Egyptian systemic lupus erythematosus patients. Patients and methods The medical records of 770 systemic lupus erythematosus patients who were followed from 2002–2015 at Kasr Alainy Hospital, Cairo University, were retrospectively reviewed. Results There were 707 (91.8%) females. The mean age at disease onset was 22.1 ± 8.6 and the disease duration was 6.1 ± 4.5 years. The main clinical manifestations were mucocutaneous (90.8% with oral ulcers affecting 52.5%), arthritis (80.3%), nephritis (67.8%), hematologic involvement (64.9%), serositis (55.2%) and neuropsychiatric manifestations (44.3%). The frequencies of antinuclear antibodies were 94.3%, anti-dsDNA 74.8%, anti-Smith 11%, anticardiolipin antibodies 29.5% and lupus anticoagulant 19.8%. Infections, predominantly bacterial, affected 337 (43.8%) patients. Thirty-three (4.3%) patients died. The main causes of death were sepsis and disease activity. The five- and 10-year survival rates for the total cohort were 97.4% and 96.3%, respectively, and were 96% and 92%, respectively for those with nephritis ( p = 0.008). Autoimmune hemolytic anemia, thrombocytopenia, elevated serum creatinine, a higher damage index, infections, a higher glucocorticoid dose and cyclophosphamide use ≥ six months were associated with an increased risk of mortality with odds ratios of 3.69, p < 0.01; 4.12, p < 0.001; 1.54, p < 0.001; 1.43, p < 0.001; 5.08, p < 0.001; 5.04, p < 0.001 and 2.25, p = 0.03, respectively. Conclusion Compared to other cohorts, a relatively lower mean age at systemic lupus erythematosus onset and higher frequencies of oral ulcers, serositis and nephritis were found.

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SLICC-based Phenotype of Juvenile Systemic Lupus Erythematosus in the Philippines

Acta Medica Philippina ◽

10.47895/amp.v54i5.1342 ◽

2020 ◽

Vol 54 (5) ◽

Author(s):

Cherica A. Tee ◽

Michael L. Tee

Keyword(s):

Lupus Erythematosus ◽

Age Of Onset ◽

The Philippines ◽

Antiphospholipid Antibody ◽

Class Iii ◽

Systemic Lupus ◽

Renal Disorder ◽

Oral Ulcers ◽

Cutaneous Rash ◽

Pediatric Sle

Objective. To describe the clinical profile of Filipino pediatric SLE patients as determined using the 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. Methods. We checked which among the SLICC criteria were fulfilled by Filipino pediatric SLE patients when weexamined them and their case records, as part of a nationwide genetic study on SLE conducted from October2015 to March 2017. Results. Ninety-seven (out of 321) who were diagnosed to have SLE before 19 years of age were evaluated.The mean age of the population at the time of evaluation was 19.8 ± 6.9 years. Females comprised 94% of ourpopulation. Mean age of onset was 14.4 ± 2.7 years, while the mean age of diagnosis was at 14.5 ± 2.6 years. Acute cutaneous rash was found in 87%; oral ulcers 65%; renal disorder 63%; non-scarring alopecia 61%; arthritis 58%; chronic cutaneous rash 36%; leukopenia 35%; hemolytic anemia 34%; serositis 25%; thrombocytopenia 23%, and neurologic disorder 8%. Anti-nuclear antibody was present in 85%; low complement 32%; anti-dsDNA 28%; direct Coombs’ 16%; antiphospholipid antibody 3%; and anti-Smith antibody 1%. Kidney biopsy was performed in only 14% (14/97) of patients, of whom 27% had class III histopathologic characteristic. Conclusions. Filipino pediatric SLE patients typically present with mucocutaneous, renal, and musculoskeletalinvolvement. Cardiopulmonary and neurologic manifestations are found to be less common among them. Finally, renal biopsy is not commonly performed among these patients.

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Prooxidant-Antioxidant Balance in Patients with Systemic Lupus Erythematosus and Its Relationship with Clinical and Laboratory Findings

Autoimmune Diseases ◽

10.1155/2016/4343514 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 1

Author(s):

Seyyed Mehdi Jafari ◽

Saeedeh Salimi ◽

Alireza Nakhaee ◽

Hamed Kalani ◽

Shima Tavallaie ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Stress Condition ◽

Clinical Manifestations ◽

Systemic Lupus ◽

Laboratory Findings ◽

Oral Ulcers ◽

The Mean ◽

Positive Significant Correlation ◽

Control Study

Aim. This study was aimed at evaluating prooxidant-antioxidant balance (PAB) in patients with systemic lupus erythematosus (SLE) and its relationship with laboratory findings and clinical manifestations.Methods. In this case-control study, 60 patients with SLE and 60 healthy individuals were enrolled. The blood samples were collected and their sera were separated. Subsequently, the prooxidant-antioxidant balance value was evaluated using PAB assay for each sample.Results. The mean of PAB values in SLE patients was significantly higher than healthy controls (147.3±42versus84.8±32.2 HK,P<0.0001). Furthermore, in SLE patients, there was a positive significant correlation between the PAB and erythrocyte sedimentation rate (ESR) (r=0.492,P<0.001). In addition, the PAB values in patients with alopecia, discoid rash, oral ulcers, arthritis, and nephritis were significantly higher than those without these manifestations.Conclusion. The findings of current study showed that the mean of PAB was significantly higher in SLE patients and PAB was correlated with ESR. Moreover increased PAB was found in SLE patients with alopecia, discoid rash, oral ulcers, arthritis, and nephritis. These findings suggest that the measurement of PAB may be useful to show oxidative stress condition in SLE patients.

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Antiphospholipid Antibodies in Pediatric Systemic Lupus Erythematosus

PEDIATRICS ◽

10.1542/peds.96.6.1040 ◽

1995 ◽

Vol 96 (6) ◽

pp. 1040-1045

Author(s):

David E. Seaman ◽

A. Vincent Londino ◽

C. Kent Kwoh ◽

Thomas A. Medsger ◽

Susan Manzi

Keyword(s):

Systemic Lupus Erythematosus ◽

Prothrombin Time ◽

Lupus Erythematosus ◽

False Positive ◽

Antiphospholipid Antibodies ◽

Clinical Manifestations ◽

Immunoglobulin M ◽

Systemic Lupus ◽

Prolonged Prothrombin Time ◽

Pediatric Sle

Objective. Antiphospholipid antibodies (aPLs) have been extensively studied in adults with systemic lupus erythematosus (SLE) and have been associated with arterial and venous thrombosis, thrombocytopenia, neurologic disorders, and recurrent fetal loss. In contrast, very little is known about the frequency and clinical significance of aPLs in pediatric SLE. This study was designed to determine the frequency of aPLs in pediatric SLE and the temporally associated clinical manifestations. Design. We studied 29 consecutive patients with onset of SLE in childhood seen in the Pediatric Rheumatology Clinic at the University of Pittsburgh, Children's Hospital, between 1985 and 1992. We defined aPL as the presence of a lupus anticoagulant (LAC), immunoglobulin G or immunoglobulin M anticardiolipin antibodies (aCLs), or a biologic false-positive serologic test for syphilis determined by a VDRL test. Clinical manifestations were temporally correlated to the presence of aPLs if they occurred within 6 months. Results. Overall, 19 (65%) of 29 children with SLE had one of the three laboratory abnormalities defining aPL. LAC was detected in 16 (62%) of 26, aCL in 18 (66%) of 27, and false-positive VDRL test results in 11 (39%) of 28. Twenty-five of the 29 patients had all three tests performed. In 10 patients, all three tests were abnormal. The presence of thrombosis in 7 patients (4 venous, 2 arterial, and 1 both) was associated with a positive aPL, specifically aCL. The presence of an aPL was significantly associated with anti-double-stranded DNA antibodies, but not with neuropsychiatric manifestations or with thrombocytopenia. The presence of an aCL was significantly associated with hemolytic anemia. A prolonged prothrombin time, in the setting of an LAC (all with a prolonged activated partial thromboplastin time), was associated with life-threatening disease in 6 of 15 patients. Conclusion. Sixty-five percent of 29 consecutive pediatric patients with SLE had evidence of aPL. The presence of aPL, specifically aCL, was significantly associated with thrombotic events. The presence of a prolonged prothrombin time in the setting of an LAC may be a marker of more serious disease in pediatric SLE.

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