Longitudinal disease- and steroid-related damage among adults with childhood-onset systemic lupus erythematosus

Heshin‐Bekenstein, Merav; Trupin, Laura; Yelin, Ed; Scheven, Emily von; Yazdany, Jinoos; Lawson, Erica

doi:10.1016/j.semarthrit.2019.05.010

Cited by 34 publications

(34 citation statements)

References 18 publications

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“…Various conflict risk factors were defined in the development of AVN in SLE patients. Some reports stated that young age is a risk factor for AVN occurrence [12][13][14]. Indeed, our patients with AVN were younger than those without AVN; however, the difference was not statistically significant.…”

Section: Discussioncontrasting

confidence: 63%

Avascular necrosis less frequently found in systemic lupus erythematosus patients with the use of alternate day corticosteroid

et al. 2020

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evaluation of symptomatic or asymptomatic patients [3]. The risk factors for AVN in SLE patients were shown to be young age, sex, cushingoid body habitus, Raynaud's phenomenon, thrombophlebitis, vasculitis, nephritis, cerebritis, interstitial pneumonitis, pleural effusion, antiphospholipid syndrome, preeclampsia, hypertension, anemia, thrombocytopenic thrombotic purpura, smoking, and migraine [4]. The objective of this study was to evaluate associated factors for the development of AVN in our SLE cohort. Materials and methodsA total of 127 consecutive SLE patients (86.6% female) were enrolled in this cross-sectional study during 10 months in our outpatient clinic. Ethical approval was received from the local ethical committee. Inclusion criteria of this study were being ≥18 years old and fulfillment of the 1997 American College of Rheumatology (ACR) revised criteria for the classification of SLE [5].Demographic data, clinical, and laboratory features were recorded from a standard questionnaire and from the patients' files. Potential associated factors of AVN such as sex, age, body mass index, age at disease diagnosis, disease duration, smoking history, hypertension, diabetes mellitus, coronary artery disease, hyperlipidemia, anemia, osteoporosis, menopause, SLE disease activity score, antiphospholipid antibodies positivity, and corticosteroid usage were noted.SLE disease activity index score (SLEDAI-2K) [6] was recorded for assessment of disease activity. SLEDAI scores were also calculated according to the situation 6 months prior to AVN diagnosis.Background/aim: Avascular necrosis (AVN) is the death of bone due to compromise of blood flow. The etiology of AVN is multifactorial; corticosteroid usage is the second most significant factor after trauma, and systemic lupus erythematosus (SLE) is the most common underlying disease. The objective of this study was to assess the factors of AVN in SLE patients. Materials and methods:The study included 127 patients with SLE who fulfilled 1997 American College of Rheumatology (ACR) revised criteria. Demographic data, age at SLE diagnosis, disease duration, disease activity, body mass index, clinical findings, antiphospholipid syndrome, steroid usage, dose and duration, comorbid diseases, and smoking history were recorded.Results: AVN was found in 11 of 127 (8.7%) SLE patients. Hyperlipidemia (P < 0.001), cushingoid body habitus (P < 0.001), and proteinuria (P = 0.013) were found at higher rates in the AVN group. All of the 11 AVN cases had osteoporosis (P < 0.02). In multivariate regression analysis, daily steroid usage was the only factor for development of AVN in SLE. Conclusion:The hypothesis of our study was that an alternate day steroid regimen may decrease AVN frequency in SLE patients.

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Section: Discussioncontrasting

confidence: 63%

Avascular necrosis less frequently found in systemic lupus erythematosus patients with the use of alternate day corticosteroid

et al. 2020

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“…The authors also demonstrated that overall the quality of life was reduced compared with the Dutch population [ 28 ]. The extent of damage and rates of accumulated damage seem to occur regardless of age in studies comparing childhood-onset and adult-onset SLE [ 44 ].…”

Section: Disease-related Damage To Other Organsmentioning

confidence: 99%

Kidney outcomes for children with lupus nephritis

et al. 2020

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Systemic lupus erythematosus is a rare lifelong multi-systemic autoimmune condition. Juvenile-onset SLE (JSLE) is recognized to have a more active disease course when compared with adult-onset disease and patients have a worse long-term survival. Kidney involvement occurs in over 50% of children and treatment decisions are guided by the histological classification. Several international groups have produced treatment protocols that rely on an intense period of immunosuppression to halt the acute kidney inflammatory process, followed by maintenance therapy with close observation for disease improvement and prompt evaluation of disease flares. A reduced glomerular filtration rate at presentation is predictive of later stage chronic kidney disease (CKD) in multivariate analysis. Kidney remission remains suboptimal with only 40–60% of patients achieving complete remission. Kidney flares are seen in over a third of patients. The rate of CKD 5 is reported to be up to 15% and the presence of lupus nephritis (LN) has an established link with an associated increase in mortality. In established kidney failure, transplantation seems to be the optimal kidney replacement modality for this group of patients, ideally after a period of disease quiescence. Modified outcome measures in clinical trials have demonstrated that biologic agents can be effective in this disease. Current biologic agents under investigation include obinutuzimab, belimumab, atacicept, anifrolumab, tocilizumab, eculizumab, dapirolizumab, and abatacept. Future research should focus on discovering early disease biomarkers, including surrogates for later cardiovascular disease, and evaluating biological agents as adjuncts to improve the rates of complete remission and subsequently influence the kidney outcome. The aim of this review article is to summarize the current kidney outcomes for this disease with a view to identifying key areas that may help to reduce the risk of long-term CKD.

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“…Patients with jSLE (as opposed to adult-onset SLE) are at increased risk of steroid-related damage. In the US Lupus Outcomes Study, a longitudinal cohort of adults with confirmed SLE, jSLE patients were more likely to report steroid-related damage (OR 1.7, 95% CI 1.1–2.8) in the adjusted analysis as compared to those with adult-onset SLE [ 131 ]. As discussed above, biologic trials in SLE have by and large been disappointing as compared to other autoimmune diseases, potentially related to the complex and heterogeneous nature of SLE, and the influence of genetic, environmental and hormonal factors.…”

Section: Introductionmentioning

confidence: 99%

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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Longitudinal disease- and steroid-related damage among adults with childhood-onset systemic lupus erythematosus

Cited by 34 publications

References 18 publications

Avascular necrosis less frequently found in systemic lupus erythematosus patients with the use of alternate day corticosteroid

Avascular necrosis less frequently found in systemic lupus erythematosus patients with the use of alternate day corticosteroid

Kidney outcomes for children with lupus nephritis

Systemic Lupus Erythematosus in Children and Young People

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