Antiphospholipid Antibodies in Pediatric Systemic Lupus Erythematosus

PEDIATRICS ◽  
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.

Childhood-Onset Systemic Lupus Erythematosus: Antiphospholipid Antibodies in 37 Patients and Their First-Degree Relatives

PEDIATRICS ◽  
1993 ◽  
Vol 92 (6) ◽  
pp. 849-853
Author(s):  
Charles Molta ◽  
Olivier Meyer ◽  
Christine Dosquet ◽  
Marcela Montes de Oca ◽  
Marie-Claude Babron ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Clinical Manifestations ◽  
Fetal Loss ◽  
Childhood Onset ◽  
Systemic Lupus ◽  
First Degree Relatives ◽  
Antiphosphatidylethanolamine Antibodies ◽  
Onset Systemic Lupus Erythematosus

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.

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.

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.

False-positive dengue IgM test result in a patient with systemic lupus erythematosus: a case report

2020 ◽  
Vol 14 (5) ◽  
pp. 209-213
Author(s):  
Supitcha Kamolratanakul ◽  
Pravinwan Thungthong ◽  
Chajchawan Nakhakes ◽  
Chokchai Kittiyanpanya ◽  
Putza Chonsawat ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
False Positive ◽  
Clinical Symptoms ◽  
Dengue Infection ◽  
Febrile Illness ◽  
Immunoglobulin M ◽  
Acute Febrile Illness ◽  
Systemic Lupus

AbstractDengue virus infection most commonly has mild-to-moderate nonspecific clinical presentations that overlap with other diseases. Dengue-specific tests are commonly used for those patients with acute febrile illness in dengue-endemic areas. There is one study in vitro that showed a false-positive dengue-immunoglobulin M (dengue IgM) test for blood from a patient with systemic lupus erythematosus (SLE). Here, we demonstrated a false-positive dengue IgM test in a patient with SLE. The patient had fever, cytopenia, and a skin rash, but her clinical variables more closely matched with the criteria for SLE than the dengue infection. Vasculitis-like-lesions supported prednisolone administration and her clinical symptoms improved. This case highlights that some patients with SLE can be misdiagnosed as having a viral infection. These two diseases have similar clinical findings, such as acute febrile illness, but they are different in terms of their treatments and disease prognosis.

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.

Clinical case of lung lesions in patient with newly diagnosed systemic lupus erythematosus

2019 ◽  
Vol 1 (9) ◽  
pp. 53-57
Author(s):  
T. N. Gavva ◽  
L. V. Kuzmenkova ◽  
Yu. N. Fedulaev ◽  
T. V. Pinchuk ◽  
D. D. Kaminer ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Clinical Case ◽  
Clinical Manifestations ◽  
Lung Damage ◽  
Newly Diagnosed ◽  
Systemic Lupus ◽  
Lung Lesions ◽  
Laboratory Parameters ◽  
Clinical Interest

A case of lung damage in systemic lupus erythematosus (SLE) in a 33-year-old woman is described. This case is of clinical interest due to the complexity of diagnosis due to the fact that SLE is a disease with diverse clinical manifestations involving many organs and systems, which often makes it difficult to timely recognize the onset of the disease. SLE still remains a challenge and requires special attention to the patient s history, clinical and laboratory parameters of the patient, as well as specific immunological examinations.

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