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.

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.

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.

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.

scholarly journals Assessment of EULAR/ACR-2019, SLICC-2012 and ACR-1997 Classification Criteria in SLE with Longstanding Disease

2021 ◽  
Vol 10 (11) ◽  
pp. 2377
Author(s):  
Berta Magallares ◽  
David Lobo-Prat ◽  
Ivan Castellví ◽  
Patricia Moya ◽  
Ignasi Gich ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Disease Duration ◽  
Renal Involvement ◽  
Damage Index ◽  
High Sensitivity ◽  
Classification Criteria ◽  
Systemic Lupus

Background: Different classification criteria for systemic lupus erythematosus (SLE) have been launched over the years. Our aim was to evaluate the performance of the EULAR/ACR-2019, SLICC-2012 and ACR-1997 classification criteria in a cohort of SLE patients with longstanding disease. Methods: Descriptive observational study in 79 patients with established and longstanding SLE. The three classification criteria sets were applied to those patients. Results: Of the 79 patients, 70 were women (88.6%), with a mean age of 51.8 ± 14 years and a mean disease duration of 15.2 ± 11.5 years. The sensitivity of the different criteria were: 51.9%, 87.3% and 86.1% for ACR-1997, SLICC-2012 and EULAR/ACR-2019, respectively. In total, 68 out of 79 patients (53.7%) met all three classification criteria; 11.4% did not meet any classification criteria and were characterized by low SLEDAI (0.6 ± 0.9), low SLICC/ACR Damage Index (0.88 ± 0.56) and fulfilling only skin domains, antiphospholipid antibodies or hypocomplementemia. To fulfill EULAR/ACR-2019 criteria was associated with low complement levels (p < 0.04), high anti-dsDNA levels (p < 0.001), presence of lupus nephritis III-IV (p < 0.05) and arthritis (p < 0.001). Conclusion: The EULAR/ACR-2019 classification criteria showed high sensitivity, similar to SLICC-2012, in SLE patients with longstanding disease. Patients with serological, articular or renal involvement are more likely to fulfill SLICC-2012 or EULAR/ACR-2019 criteria.

The Relationship Between Systemic Lupus Erythematosus Activity and Persistent Positive Antiphospholipid Antibodies

2018 ◽  
Vol 14 (2) ◽  
pp. 145-152 ◽  
Author(s):  
Zhaleh Shariati Sarabi ◽  
Maryam Sahebari ◽  
Ali Etemad Rezaie ◽  
Mohammad Taghi Norouzi ◽  
Kamila Hashemzadeh ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibodies ◽  
Systemic Lupus ◽  
Systemic Lupus Erythematosus Activity ◽  
The Relationship
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