ANTIPHOSPHOLIPID ANTIBODIES IN PAEDIATRIC SYSTEMIC LUPUS ERYTHEMATOSUS, JUVENILE CHRONIC ARTHRITIS AND OVERLAP SYNDROMES: SLE PATIENTS WITH BOTH LUPUS ANTICOAGULANT AND HIGH-TITRE ANTICARDIOLIPIN ANTIBODIES ARE AT RISK FOR CLINICAL MANIFESTATIONS RELATED TO THE ANTIPHOSPHOLIPID SYNDROME

Objective. Antiphospholipid antibodies (aPL) are noted with increased frequency in patients with systemic lupus erythematosus (SLE). The main manifestations found to be associated with aPL are arterial and venous thrombotic events, thrombocytopenia, and recurrent pregnancy loss This study is an attempt to define the incidence of aPL in patients with childhood-onset SLE and in their relatives and to correlate their presence with clinical manifestations, and especially, to evaluate the risk of thrombosis in aPL-positive subjects. Methodology. We studied 37 unrelated patients and 107 of their first-degree relatives. VDRL, IgG and IgM anticardiolipin, and IgG antiphosphatidylethanolamine antibodies were studied in all probands during periods of clinical remission and in first-degree relatives at the time of interview. Lupus anticoagulant had only been studied in probands during an SLE flare-up. Results. Thirty-eight percent of probands and 19% of relatives were positive for at least one aPL, with little over-lap between the different aPL studied. -No aPL-negative proband developed thrombosis. Two of the aPL-positive probands had thrombotic events before testing, and a third one showed thrombosis after testing. Only two probands had high levels of IgG aCL and showed thrombosis. The occurrence of aPL positivity in relatives was not always related to its presence in probands. None of the aPL-positive relatives had hadthrombosis, but recurrent fetal loss was noted in one aPL-positive mother with SLE. Although there was a high frequency of SLE, SLE-like disease, auto-immune disorders or positive serological findings for lupus in first-degree relatives, many of these relativew did not test positive for aPL. Conclusion. The high levels of IgG aCL may be considered a risk factor for thrombosis. Findings in relatives suggest a multifactorial origin for autoimmune disease and antibody production.

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Antiphospholipid antibodies in 57 children and adolescents with systemic lupus erythematosus

Revista do Hospital das Clínicas ◽

10.1590/s0041-87812003000300005 ◽

2003 ◽

Vol 58 (3) ◽

pp. 157-162 ◽

Cited By ~ 6

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.

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Antiphospholipid syndrome accompanying systemic lupus erythematosus

Medicinski pregled ◽

10.2298/mpns0204089m ◽

2002 ◽

Vol 55 (3-4) ◽

pp. 89-96 ◽

Cited By ~ 2

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.

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Stroke in an Early Adolescent with Systemic Lupus Erythematosus and Coexistent Antiphospholipid Antibodies

PEDIATRICS ◽

10.1542/peds.90.1.96 ◽

1992 ◽

Vol 90 (1) ◽

pp. 96-99

Author(s):

DAVID D. DUNGAN ◽

M. SUSAN JAY

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Anticardiolipin Antibodies ◽

Early Adolescent ◽

Systemic Lupus ◽

Phosphodiester Group ◽

Long Time ◽

Reaginic Antibodies ◽

Activator Complex

Thromboembolic events in systemic lupus erythematosus (SLE) are an occasional but significant occurrence. Cerebrovascular disease in adults with SLE has been well described.1-4 Antiphospholipid antibodies, such as lupus anticoagulant, an immunoglobulin directed against the platelet phospholipid component of the prothrombin activator complex, and the false-positive serologic tests for syphilis, which detect reaginic antibodies that react with the purified beef cardiolipin substrate of these assays, have been reported for a long time in association with these events.5-9 More recently, development of an assay for anticardiolipin antibodies, immunoglobulins directed against the phosphodiester group of negatively charged phospholipids, has led to wider exploration of this subset of antiphospholipid antibodies and their relation to thrombosis.10,11

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Connective tissue disease

Oxford Handbook of Clinical Immunology and Allergy ◽

10.1093/med/9780199603244.003.0012 ◽

2013 ◽

pp. 257-300

Author(s):

Gavin P Spickett

Keyword(s):

Rheumatoid Arthritis ◽

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Juvenile Chronic Arthritis ◽

Chronic Arthritis ◽

Still’S Disease ◽

Systemic Lupus ◽

Still's Disease ◽

Adult Still's Disease ◽

Immunological Tests

Rheumatoid arthritis: aetiology, clinical features, and immunopathology Rheumatoid arthritis: immunological tests, treatment, and childhood RhA Juvenile chronic arthritis (JCA) and Still’s disease Adult Still’s disease Ankylosing spondylitis (AS) and related spondyloarthropathies SAPHO syndrome Psoriatic arthritis Reactive arthritis (including Reiter’s syndrome) Systemic lupus erythematosus (SLE) and variants...

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Audiovestibular manifestations of the antiphospholipid syndrome

The Journal of Laryngology & Otology ◽

10.1017/s0022215100125848 ◽

1994 ◽

Vol 108 (1) ◽

pp. 57-59 ◽

Cited By ~ 25

Author(s):

Tim Vyse ◽

Linda M. Luxon ◽

Mark J. Walport

Keyword(s):

Systemic Lupus Erythematosus ◽

Antiphospholipid Syndrome ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Anticardiolipin Antibodies ◽

The Other ◽

Primary Antiphospholipid Syndrome ◽

Systemic Lupus

AbstractWe report on two patients who have high titres of antiphospholipid antibodies, both of whom had acute audiovestibular failure. One of the patients had systemic lupus erythematosus. The other patient had primary antiphospholipid syndrome: audiovestibular symptoms have not been reported in this condition. The occurrence of acute audiovestibular failure in the primary antiphospholipid syndrome raises the question as to whether patients presenting with acute deafness or vestibular disturbance should be screened for the presence of anticardiolipin antibodies.

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CLINICAL AND IMMUNOLOGICAL CORRELATIONS IN PATIENTS WITH SYSTEMICLUPUS ERYTHEMATOSUS WITH ANTIPHOSPHOLIPID ANTIBODY SYNDROME

HERALD of North-Western State Medical University named after I I Mechnikov ◽

10.17816/mechnikov2017937-11 ◽

2017 ◽

Vol 9 (3) ◽

pp. 7-11 ◽

Cited By ~ 1

Author(s):

E A Belolipetskaia ◽

I B Beliaeva ◽

V I Mazurov ◽

E A Trofimov ◽

S V Lapin

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Annexin V ◽

Anticardiolipin Antibodies ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Systemic Lupus ◽

Glycoprotein I ◽

Threefold Increase

Antiphospholipid antibodies (aPL): lupus anticoagulant (LA), anticardiolipin antibodies (aCL) and anti-β2-glycoprotein I antibodies (anti-β2GPI) are found in 12 to 44% of systemic lupus erythematosus (SLE) patients. On average, antiphospholipid antibody syndrome (APS) develops in50% of aPL-positive patients with SLE. The seronegative APS is characterized by the absence of the diagnostic levels of "classical" aPL and by the presence of non-criteria aPL: antibodies against pro- thrombin (aPT), antibodies against annexin V, antibodies against phosphatidylethanoamine (aPE), antibodies to phosphatidylserine/prothrombin complex (aPS-PT) and antibodies against negatively charged phospholipids. The presence of four antibodies (LA + aCT + anti-β2GPI + aPT) is associated with a threefold increase in the risk of thrombosis. The presence of aCL and anti-β2GPI in SLE patients with APS and recurrent thromboses is associated with the HLA dRB1 * 0402.

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Autoimmune Thyroid Disorders in Juvenile Chronic Arthritis and Systemic Lupus Erythematosus

Rheumaderm - Advances in Experimental Medicine and Biology ◽

10.1007/978-1-4615-4857-7_8 ◽

1999 ◽

pp. 55-60 ◽

Cited By ~ 45

Author(s):

D. Mihailova ◽

R. Grigorova ◽

B. Vassileva ◽

G. Mladenova ◽

N. Ivanova ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Juvenile Chronic Arthritis ◽

Chronic Arthritis ◽

Thyroid Disorders ◽

Systemic Lupus ◽

Autoimmune Thyroid

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The Relationship of Antiphospholipid Antibodies to Thromboembolic Disease in Systemic Lupus Erythematosus: A Cross-Sectional Study

Thrombosis and Haemostasis ◽

10.1055/s-0038-1646452 ◽

1991 ◽

Vol 66 (05) ◽

pp. 520-524 ◽

Cited By ~ 82

Author(s):

A A Long ◽

J S Ginsberg ◽

P Brill-Edwards ◽

M Johnston ◽

C Turner ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Antiphospholipid Antibodies ◽

Cross Sectional Study ◽

Anticardiolipin Antibodies ◽

Thromboembolic Events ◽

Sectional Study ◽

Systemic Lupus ◽

Cross Sectional ◽

Relationship Of

SummaryIn order to determine whether an association exists between antiphospholipid antibodies (APLA) and thromboembolic events in patients with systemic lupus erythematosus (SLE), we performed a cross-sectional study of consecutive unselected SLE patients. The occurrence of previous thromboembolic events was determined by investigators blinded to the APLA status of the patients by critical review of objective tests that had been performed at the time of symptomatic presentation and by performing venous Doppler ultrasound of the legs to elicit venous reflux as an indication of previous venous thrombosis. The presence of APLA was determined by coagulation assays for the lupus anticoagulant (LA) using five tests with well-defined control ranges and by ELISA assay for anticardiolipin antibodies (ACLA). These tests were measured on two separate occasions. The results of the study demonstrate a statistically significant association between persistently abnormal ACLA assays and thromboembolic events and a non-significant trend between persistently abnormal LA and thromboembolic events. Transient abnormalities of LA and ACLA were less strongly associated with thromboembolic events. We conclude that in patients with SLE, there is a significant association between thromboembolism and APLA.

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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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