Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey

Balcı, Sibel; Ekinci, Rabia Miray Kışla; Bayazıt, Aysun Karabay; Melek, Engin; Doğruel, Dilek; Altıntaş, Derya Ufuk; Yılmaz, Mustafa

doi:10.1007/s10067-019-04433-4

Cited by 24 publications

(28 citation statements)

References 43 publications

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“…Fifty-eight jSLE patients, who were diagnosed between January 2006 and February 2019 prior to their 18 th birthday included in this study. We have extended our previous reported data focusing more on AVN and organ damage by including more jSLE patients 1 .…”

Section: Methodsmentioning

confidence: 78%

“…Systemic lupus erythematosus (SLE) is a multisystemic autoimmune disease with various clinical manifestations 1,2 . Among all SLE patients, 15-20% have a disease onset in childhood namely juvenile SLE (jSLE) 3 .…”

Section: Introductionmentioning

confidence: 99%

“…Avascular necrosis is a debilitating disorder that characterized by interruption of the blood supply which leads to death of the bone marrow and trabecular bone, presenting with joint pain, bone destruction, and loss of function 8 . It is a well-known complication of SLE since its first determination in 1960 and has been reported in 1.4-40% of jSLE patients [1][2][3][4][9][10][11][12][13][14] . The most common affected sites are femoral head, femoral condyles, proximal tibia, and the bones of foot and ankle 8 .…”

Section: Introductionmentioning

confidence: 99%

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Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Balcı

Ekinci

Pişkin

et al. 2020

Cukurova Medical Journal

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Öz Purpose: Avascular necrosis (AVN) is a debilitating complication of juvenile systemic lupus erythematosus (jSLE). The aim of this study was to evaluate the frequency and clinical characteristics of patients with AVN in jSLE from a single center. Material and Methods: Fifty-eight jSLE patients diagnosed according to the American College of Rheumatology classification criteria were included in this retrospective study. Disease activity of jSLE patients was measured by SLE Disease Activity Index-2K (SLEDAI-2K), organ damage was determined by the pediatric version of the systemic lupus international collaborating clinics/American College of Rheumatology damage index (pedSDI) at last visit. Results: Among 58 jSLE patients, the female patients accounted for 86.2% (n=50). Mean baseline SLEDAI-2K score was 21.47±8.96. The number of patients with at least one damage item of pedSDI was 24 (41.4%), in which 5 patients (8.6%) had AVN. There were no statistical differences between the groups except baseline complement 3 (C3) level was significantly lower in patients with AVN. Conclusion: Baseline low C3 level might be a predictor for AVN development in jSLE patients.There is unmet need for multicenter studies investigating possible risk factors of AVN in jSLE patients. Amaç: Avasküler nekrozis (AVN), çocukluk çağı başlangıçlı sistemik lupus eritematozusun (jSLE) yıkıcı bir komplikasyonudur. Tek merkezden yapılan bu çalışmanın amacı jSLE hastalarında AVN sıklığını ve bu hastaların klinik özelliklerini belirlemektir. Gereç ve Yöntem: Amerikan Romatoloji Koleji'nin sınıflama kriterlerine göre tanı almış 58 jSLE hastası bu geriye dönük çalışmaya dahil edildi. jSLE hastalarının hastalık aktiviteleri SLE hastalık aktivite indeksi baz alınarak (SLEDAI-2K) ölçüldü. Organ hasarı Amerikan Romatoloji Koleji'nin SLE hastalarında uyguladığı hasar indeksinin çocukluklardaki versiyonu kullanılarak son muayenede belirlendi. Bulgular: Elli sekiz jSLE hastasının %86,2 (50)'si kız hastaydı. Ortalama bazal SLEDAI-2K skoru 21,47±8,96 idi. En az bir organda hasarı olan hasta sayısı 24 (%41,4) idi, bunlardan 5 tanesinde (%8,6) AVN vardı. AVN olan ve olmayan hastalar arasında çeşitli değikenlere göre istatistiksel anlamlı bir farklılık saptanmadı. Sadece bazal kompleman 3 (C3) düzeyi AVN hastalarında, AVN olmayan hastalara göre anlamlı olarak düşüktü. Sonuç: Bazal düşük C3 düzeyi jSLE hastalarında AVN gelişimini tahmin etmede ön görücü olabilir. jSLE hastalarında AVN gelişimi için muhtemel risk faktörlerini inceleyen çok merkezli çalışmalara ihtiyaç vardır.

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Section: Methodsmentioning

confidence: 78%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Balcı

Ekinci

Pişkin

et al. 2020

Cukurova Medical Journal

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“…Several endogenous and exogenous factors are held accountable in the pathogenesis of SLE. 1–3 The condition involves the generation of self-reactive antibodies, increased antigenic load, regulatory T-cell dysfunction and polyclonal B-cell activation. 4–8 The genetic, environmental and infectious processes, especially viral infections, appear to play a role in the immune dysregulation.…”

Section: Introductionmentioning

confidence: 99%

Epstein–Barr virus, cytomegalovirus and BK polyomavirus burden in juvenile systemic lupus erythematosus: correlation with clinical and laboratory indices of disease activity

Aygün

Kuşkucu

Şahin

et al. 2020

Lupus

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Objectives Clinical and laboratory investigations have revealed that Epstein–Barr virus (EBV) is involved in altered immunological response of systemic lupus erythematosus (SLE). Higher seroprevalence rates of anti-EBV antibodies and increased viral load are demonstrated in adult SLE patients. The prevalence of BK polyomavirus (BKV) reactivation is also suggested to be higher in SLE. Herein, we aimed to evaluate the immune response of children with SLE to EBV antigens in addition to EBV and BKV DNA. We also tried to evaluate whether these serological results differ from another connective tissue disease – juvenile systemic sclerosis (jSS) – and healthy individuals. Methods Serum levels of EBV early antigen diffuse (EA-D) IgG, EBV nuclear antigen-1 IgG, EBV viral capsid antigen (VCA), cytomegalovirus (CMV) IgG, EBV DNA, CMV DNA and urinary BKV DNA were evaluated in healthy controls and in patients with a diagnosis of juvenile SLE (jSLE) and jSS. Results A total of 70 jSLE patients, 14 jSS patients and 44 sex-matched healthy individuals were involved in the study. EBV VCA was positive in 84.2% of jSLE patients, 85.7% of jSS patients and 36.3% of healthy controls. EBV EA-D IgG positivity was significantly higher in jSLE patients compared to jSS patients and healthy controls (20% vs. 7.1% and 0%, p = 0.005). EBV VCA positivity was associated with malar rash and immunological disorder, but there was no statistical significance in other antibody positivity in terms of clinical and haemogram findings and autoantibody positivity. CMV DNA positivity was present in only 2.8% of jSLE patients. None of the jSS patients or the healthy controls had CMV DNA positivity. EBV DNA and BKV DNA were also negative in all three groups. Conclusion The results of our study assume a relationship between SLE and EBV, but we could not demonstrate an association between CMV and BKV. The negative DNA results in contrast to serological positivity can be interpreted as an altered and impaired immune system and increased viral susceptibility. These results suggest that EBV contributes to disease continuity, even if it does not directly cause development.

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“…1 Disease incidence, severity, organ involvement, mortality and morbidity rate of juvenile SLE (jSLE) vary among ethnic groups. 2,3 The course of jSLE is more severe than adult-onset SLE and jSLE requires more intensive treatment to prevent higher rates of mortality. 4 However, the mortality of jSLE patients has considerably decreased over the past decades, presumably in response to early diagnosis and better treatment modalities.…”

mentioning

confidence: 99%

Retrospective Analysis of the Factors Affecting Growth Parameters in Turkish Children With Systemic Lupus Erythematosus

et al. 2020

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Objectives: This study aims to investigate the growth parameters in Turkish children with systemic lupus erythematosus (SLE) and to compare these growth parameters according to the presence or absence of cyclophosphamide, rituximab treatment, cumulative corticosteroid dose, proliferative nephritis, and the last visit disease activity and damage index. Patients and methods: Medical data, growth parameters including z-scores for weight, height, and body mass index and parent-adjusted height z-scores of 45 juvenile SLE (jSLE) patients (5 males, 40 females; mean age 12.3±3.2 years; range 7.1 to 18 years) were retrospectively evaluated. Growth parameters were calculated by anthropometric references in Turkish children. The disease activity was assessed by the SLE Disease Activity Index 2000 (SLEDAI-2K). The disease outcome was measured by the Pediatric version of

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Juvenile systemic lupus erythematosus: a single-center experience from southern Turkey

Cited by 24 publications

References 43 publications

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Çocukluk çağı başlangıçlı sistemik lupus eritematozus hastalarında avasküler nekrozis sıklığı

Epstein–Barr virus, cytomegalovirus and BK polyomavirus burden in juvenile systemic lupus erythematosus: correlation with clinical and laboratory indices of disease activity

Retrospective Analysis of the Factors Affecting Growth Parameters in Turkish Children With Systemic Lupus Erythematosus

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