Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options

Smith, Eve; Lythgoe, Hanna; Midgley, Angela; Beresford, Michael W.; Hedrich, Christian M.

doi:10.1016/j.clim.2019.108274

Cited by 129 publications

(142 citation statements)

References 211 publications

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“…Similar to adult studies, jSLE was found to be more prevalent in patients from minority ethnic backgrounds, compared to the UK national census figures for prevalence of ethnic minorities in the population as a whole, with 51% of UK jSLE Cohort Study participants being Caucasian, compared with 86% of the UK population as a whole [29]. Of interest, the present study also showed higher numbers of male patients and less ANA and/or anti-dsDNA positivity among Caucasians (as compared to patients from minority ethnic backgrounds), which may be due to the presence of more 'atypical' and/or 'monogenic' disease in Caucasians [2,4,29].…”

Section: Impact Of Age On Disease Presentationsupporting

confidence: 47%

“…Several factors, including familial clusters, aforementioned ethnicity distribution with over-representation with minority ethnic groups, age-specific differences in clinical and laboratory phenotypes when compared to adults, and more severe and difficult-to-treat organ manifestations suggest variable pathogenic mechanisms between age groups, and a stronger involvement of genetic factors in childhood. However, especially as genetically identical monozygotic twins only exhibit disease concordance in 40-60% or all cases, additional factors appear to be involved in the pathophysiology of jSLE, further complicating the situation [2,4,[30][31][32].…”

Section: The Molecular Pathophysiology Of Jslementioning

confidence: 99%

“…Juvenile-onset systemic lupus erythematosus (jSLE) is a rare but severe multisystem autoimmune/inflammatory disease that can affect any organ system and cause significant damage, disability and/or death. It is defined by disease onset before the age of 18 and affects approximately 15-20% of SLE patients [1][2][3][4][5]. The incidences of jSLE ranges between 0.36 and 2.5 per 100,000 children, with a prevalence of 1.89-34.1 per 100,000 [6][7][8][9][10].…”

Section: Introductionmentioning

confidence: 99%

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Systemic Lupus Erythematosus in Children and Young People

2021

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Purpose of Review Juvenile-onset systemic lupus erythematosus ((j)SLE) is an autoimmune/inflammatory disease that results in significant damage and disability. When compared to patients with disease onset in adulthood, jSLE patients exhibit increased disease activity, damage and require more aggressive treatments. This manuscript summarises age-specific pathogenic mechanisms and underscores the need for age group–specific research, classification and treatment. Recent Findings Genetic factors play a significant role in the pathophysiology of jSLE, as > 7% of patients develop disease as a result of single gene mutations. Remaining patients carry genetic variants that are necessary for disease development, but require additional factors. Increased ‘genetic impact’ likely contributes to earlier disease onset and more severe phenotypes. Epigenetic events have only recently started to be addressed in jSLE, and add to the list of pathogenic mechanisms that may serve as biomarkers and/or treatment targets. To allow meaningful and patient-oriented paediatric research, age-specific classification criteria and treatment targets require to be defined as currently available tools established for adult-onset SLE have limitations in the paediatric cohort. Summary Significant progress has been made in understanding the pathophysiology of jSLE. Meaningful laboratory and clinical research can only be performed using age group–specific tools, classification criteria and treatment targets.

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Section: Impact Of Age On Disease Presentationsupporting

confidence: 47%

Section: The Molecular Pathophysiology Of Jslementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Systemic Lupus Erythematosus in Children and Young People

2021

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“…4 The worldwide prevalence of paediatric SLE ranges from 1.89 to 34.1 per 100,000 children. 5 The prevalence of paediatric SLE is more common in non-Caucasian ethnicities, and renal involvement is predominant in children and Asian heritage. The five-year survival rate in paediatric-onset SLE among Thai children has been reported to be 76.5%.…”

Section: Introductionmentioning

confidence: 99%

Photoprotective habits in children with systemic lupus erythematosus

Janthongsri

Wisuthsarewong

Nitiyarom

2020

Lupus

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Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease that can involve multiple organ systems. Exposure to ultraviolet radiation (UVR) can exacerbate pre-existing SLE, and can even induce systemic manifestations. This study aimed to investigate the photoprotective habits of children with SLE and the factors that significantly influence those photoprotective habits. Methods This questionnaire-based cross-sectional study included paediatric SLE patients being treated at the Department of Paediatrics at Siriraj Hospital, Mahidol University, between September 2018 and September 2019. Data were obtained from medical records and a face-to-face interview. Results Ninety-six patients were enrolled, with a female-to-male ratio of 8:1. The mean age of patients at enrollment was 13.7 ± 2.4 years. Of the 96 patients, 70 (72.9%) reported being directly exposed to sunlight for less than two hours per day, but 39% of patients spent time in the sun during the peak hours of UVR. Up to 95% of patients used sunscreen. However, only 64% of patients applied it every day, and only 35% of patients used an adequate amount of sunscreen. Girls were significantly more likely to apply sunscreen every day than boys were ( p = 0.041). SLE patients with a shorter disease duration had significantly greater exposure to sunlight than patients with a disease duration of more than four years ( p = 0.040). Conclusion Sunscreen was the most common photoprotective method. However, most patients used sunscreen inappropriately. A shorter disease duration was significantly associated with more sunlight exposure. Regular evaluation and emphasis of the importance of photoprotection should be encouraged among paediatric SLE.

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“…Lupus nephritis (LN) is a common manifestation of SLE. In the early stage of SLE, LN may show slight abnormalities in urine(microhematuria and /or albuminuria and /or leukuria) [2][3][4][5] , or nephrotic syndrome, hypertension, or renal failure [6][7] . In this case, the child was diagnosed with nephrotic syndrome and renal biopsy revealed lupus nephritis.…”

Section: Discussionmentioning

confidence: 99%

The Truth Behind Hematuria

2020

Preprint

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Abstract Background: Systemic lupus erythematosus(SLE) is an autoimmune disease involving multiple systems with various clinical manifestations,renal involvement is common, but intracardiac thrombus is rarely reported as a complication of antiphospholipid syndrome. Drug anticoagulation is the first choice, and surgical treatment is performed in severe cases, we report the case to improve clinicians' understanding of disease diagnosis.Case presentation: A 8-year-old girl was admitted to our hospital because of left costal pain, hematuria and fever.She had obvious edema, urinary protein 3+and occult blood 3+,urinary protein 3.2g/24h, albumin 17.6g/L, total cholesterol 7.21mmol/L, consistent with diagnosis of nephrotic syndrome. Continued to track the etiology of nephrotic syndrome and performed a renal biopsy, dsDNA 1:10 positive,low C3, low platelets and hemoglobin, anti-cardiolipin IgM 12U/ml, anti-β2-glycoprotein I 223R/ml, renal pathology suggested lupus nephritis, finally diagnosed as systemic lupus erythematosus, secondary anticardiolipin syndrome, lupus nephritis. The patient was treated with hormone and immunosuppressant. Sixteen weeks later, urinary protein 1+,the quantity of urine protein was less than 0.5g/d. Echocardiography showed that the mass in the right atrium was thrombosis. Heparin anticoagulant therapy is effective.Conclusion: Systemic lupus erythematosus can involve multiple systems,various complications, it is rare to have thrombus in the right atrium as a complication of antiphospholipid syndrome, early diagnosis and treatment is the key to improve the prognosis of children.

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Juvenile-onset systemic lupus erythematosus: Update on clinical presentation, pathophysiology and treatment options

Cited by 129 publications

References 211 publications

Systemic Lupus Erythematosus in Children and Young People

Systemic Lupus Erythematosus in Children and Young People

Photoprotective habits in children with systemic lupus erythematosus

The Truth Behind Hematuria

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