Childhood Systemic Lupus Erythematosus: Presentation, management and long-term outcomes in an Australian cohort

Lupus ◽  
2022 ◽  
pp. 096120332110697
Author(s):  
Megan P Cann ◽  
Anne M Sage ◽  
Elizabeth McKinnon ◽  
Senq-J Lee ◽  
Deborah Tunbridge ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Incidence Rate ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus ◽  
End Organ Damage ◽  
American College Of Rheumatology

Objectives Systemic Lupus Erythematosus (SLE) is a serious autoimmune disease often resulting in major end-organ damage and increased mortality. Currently, no data exists focussing on the presentation, long-term management and progression of SLE in the Australian paediatric population. We conducted the first Australian longitudinal review of childhood SLE, focussing on response to treatment and outcomes. Methods Detailed clinical and laboratory data of 42 children diagnosed with SLE before 16 years from 1998 to 2018 resident in Western Australia was collected. Data was collected at diagnosis and key clinical review time points and compared using the Systemic Lupus Collaborating Clinics (SLICC) and Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) criteria. End organ damage was assessed against Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI). Incidence rates of disease complications and end organ damage were determined. Results Of the 42 children, 88% were female with average age at diagnosis of 12.5 years. Indigenous Australians were over represented with an incidence rate 18-fold higher than non-Indigenous, although most children were Caucasian, reflecting the demographics of the Australian population. Median duration of follow-up was 4.25 years. On final review, 28.6% had developed cumulative organ damage as described by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (incidence rate: 0.08/PY (95% CI 0.04–0.14)), and one child died. Twenty-nine children had renal involvement (incidence rate: 0.38/PY (95% CI 0.26–0.56)). Of the 27 patients with biopsy proven lupus nephritis, 70% had Class III or IV disease. Average length of prednisolone use from diagnosis was 32.5 months. Hydroxychloroquine ( n = 36) and mycophenolate mofetil ( n =21) were the most widely used steroid sparing agents. 61.9% received rituximab and/or cyclophosphamide. Conclusion This is the first longitudinal retrospective review of Australian children with SLE, with a markedly higher incidence in Indigenous children. Although improving, rates of end organ complications remain high, similar to international cohort outcomes. Longitudinal multi-centre research is crucial to elucidate risk factors for poor outcomes, and identifying those warranting early more aggressive therapy.

scholarly journals Organ damage in patients treated with belimumab versus standard of care: a propensity score-matched comparative analysis

2019 ◽  
Vol 78 (3) ◽  
pp. 372-379 ◽  
Author(s):  
Murray B Urowitz ◽  
Robert L Ohsfeldt ◽  
Ronald C Wielage ◽  
Kari A Kelton ◽  
Yumi Asukai ◽  
...  
Keyword(s):  
Propensity Score ◽  
Lupus Erythematosus ◽  
Damage Index ◽  
Standard Of Care ◽  
Organ Damage ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
Damage Progression

ObjectivesThe study (206347) compared organ damage progression in patients with systemic lupus erythematosus (SLE) who received belimumab in the BLISS long-term extension (LTE) study with propensity score (PS)-matched patients treated with standard of care (SoC) from the Toronto Lupus Cohort (TLC).MethodsA systematic literature review identified 17 known predictors of organ damage to calculate a PS for each patient. Patients from the BLISS LTE and the TLC were PS matched posthoc 1:1 based on their PS (±calliper). The primary endpoint was difference in change in Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score from baseline to 5 years.ResultsFor the 5- year analysis, of 567 (BLISS LTE n=195; TLC n=372) patients, 99 from each cohort were 1:1 PS matched. Change in SDI score at Year 5 was significantly lower for patients treated with belimumab compared with SoC (−0.434; 95% CI –0.667 to –0.201; p<0.001). For the time to organ damage progression analysis (≥1 year follow-up), the sample included 965 (BLISS LTE n=259; TLC n=706) patients, of whom 179 from each cohort were PS-matched. Patients receiving belimumab were 61% less likely to progress to a higher SDI score over any given year compared with patients treated with SoC (HR 0.391; 95% CI 0.253 to 0.605; p<0.001). Among the SDI score increases, the proportion of increases ≥2 was greater in the SoC group compared with the belimumab group.ConclusionsPS-matched patients receiving belimumab had significantly less organ damage progression compared with patients receiving SoC.

scholarly journals Osteopontin and Disease Activity in Patients with Recent-onset Systemic Lupus Erythematosus: Results from the SLICC Inception Cohort

2019 ◽  
Vol 46 (5) ◽  
pp. 492-500 ◽  
Author(s):  
Lina Wirestam ◽  
Helena Enocsson ◽  
Thomas Skogh ◽  
Leonid Padyukov ◽  
Andreas Jönsen ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Damage Index ◽  
Organ Damage ◽  
Inception Cohort ◽  
Systemic Lupus ◽  
Recent Onset ◽  
Damage Accrual

Objective.In cross-sectional studies, elevated osteopontin (OPN) levels have been proposed to reflect, and/or precede, progressive organ damage and disease severity in systemic lupus erythematosus (SLE). We aimed, in a cohort of patients with recent-onset SLE, to determine whether raised serum OPN levels precede damage and/or are associated with disease activity or certain disease phenotypes.Methods.We included 344 patients from the Systemic Lupus International Collaborating Clinics (SLICC) Inception Cohort who had 5 years of followup data available. All patients fulfilled the 1997 American College of Rheumatology (ACR) criteria. Baseline sera from patients and from age- and sex-matched population-based controls were analyzed for OPN using ELISA. Disease activity and damage were assessed at each annual followup visit using the SLE Disease Activity Index 2000 (SLEDAI-2K) and the SLICC/ACR damage index (SDI), respectively.Results.Compared to controls, baseline OPN was raised 4-fold in SLE cases (p < 0.0001). After relevant adjustments in a binary logistic regression model, OPN levels failed to significantly predict global damage accrual defined as SDI ≥ 1 at 5 years. However, baseline OPN correlated with SLEDAI-2K at enrollment into the cohort (r = 0.27, p < 0.0001), and patients with high disease activity (SLEDAI-2K ≥ 5) had raised serum OPN (p < 0.0001). In addition, higher OPN levels were found in patients with persistent disease activity (p = 0.0006), in cases with renal involvement (p < 0.0001) and impaired estimated glomerular filtration rate (p = 0.01).Conclusion.The performance of OPN to predict development of organ damage was not impressive. However, OPN associated significantly with lupus nephritis and with raised disease activity at enrollment, as well as over time.

scholarly journals SAT0232 PERCEPTION OF THE DISEASE IN PATIENTS WITH EARLY SYSTEMIC LUPUS ERYTHEMATOSUS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1059.3-1059
Author(s):  
M. Garabajiu ◽  
L. Mazur-Nicorici ◽  
T. Rotaru ◽  
V. Salaru ◽  
S. B. Victoria ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Systemic Lupus Erythematosus ◽  
Disease Activity ◽  
Lupus Erythematosus ◽  
Disease Duration ◽  
Activity Index ◽  
Damage Index ◽  
Organ Damage ◽  
Systemic Lupus

Background:Systemic lupus erythematosus is an autoimmune disease with a major impact on patient’s quality of life.Objectives:To evaluate patient’s attitude toward early disease and factors that influence it.Methods:Performed case-control study included SLE patients that fulfilled SLICC, 2012 classification criteria. The research included two groups of patients: early SLE – 1stgroup (disease duration ≤24 months) and non-early SLE – 2ndgroup control (disease duration >24 months). The pattern of the disease activity was assessed by patient global assessment (PGA), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2K) and Systemic Lupus Activity Measure (SLAM), for SLE activity, SLICC/ACR Damage Index (DI) for disease irreversible changes and SF-8 for the Quality of Life (QoL).Results:A total of 101 SLE patients with 34 in the 1stgroup (early SLE) and 67 in the 2ndgroup (non-early SLE) was analyzed. The disease activity showed high disease activity in both groups by SLEDAI (7,02±4,16 and 6,26±4,43 points, p>0,05) and SLAM (7,47±4,40 and 7,31±4,10 points, p>0,05) such as (46,97±19,39 vs 47,98±22,41 points). The QoL was appreciated as low, by both components (mental and physical), in groups. The damage index was higher in the 2nd group (0,23±0,43 and 1,07±1,29, p<0,001), which can be explained by the development of irreversible changes with the increase of disease duration.The PGA in early SLE was influenced by subjective symptoms contained in SLAM index (r=0,48, p<0,05), such as fatigue and depression, and the level of the quality of life (r=0,65, p<0,001). Meantime, PGA in patients with longer disease duration (>2 years), was influenced by the presence of organ damage by SLICC/ACR DI (0,23, p<0,05) and objective findings of the disease activity contained in SLEDAI (r=0,33, p<0,005) and SLAM (0,44, p<0,001).Conclusion:The disease recognition in patients with early SLE was determined by subjective and psycho-emotional signs, while in patients with longer disease duration it was influenced by organ damage and complications.References:no referencesDisclosure of Interests:None declared

scholarly journals Association between organ damage and mortality in systemic lupus erythematosus: a systematic review and meta-analysis

BMJ Open ◽  
2020 ◽  
Vol 10 (5) ◽  
pp. e031850 ◽  
Author(s):  
Irene B Murimi-Worstell ◽  
Dora H Lin ◽  
Henk Nab ◽  
Hong J Kan ◽  
Oluwadamilola Onasanya ◽  
...  
Keyword(s):  
Systematic Review ◽  
Systemic Lupus Erythematosus ◽  
Latin American ◽  
Lupus Erythematosus ◽  
Meta Analysis ◽  
Damage Index ◽  
Organ Damage ◽  
Cochrane Library ◽  
Systemic Lupus ◽  
Cross Sectional

ObjectiveAt least half of patients with systemic lupus erythematosus (SLE) develop organ damage as a consequence of autoimmune disease or long-term therapeutic steroid use. This study synthesised evidence on the association between organ damage and mortality in patients with SLE.DesignSystematic review and meta-analysis.MethodsElectronic searches were performed in PubMed, Embase, Cochrane Library and Latin American and Caribbean Health Sciences Literature for observational (cohort, case-control and cross-sectional) studies published between January 2000 and February 2017. Included studies reported HRs or ORs on the association between organ damage (measured by the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI) score) and mortality. Study quality was assessed using the modified Newcastle-Ottawa assessment. Pooled HRs were obtained using the DerSimonian and Laird random-effects model. Heterogeneity was assessed using the Cochrane Q (Q) and I2 statistics.ResultsThe search yielded 10 420 articles, from which 21 longitudinal studies were selected. Most studies (85%) were of high quality. For 10 studies evaluating organ damage (SDI) as a continuous variable and reporting HR as a measure of association, a 1-unit increase in SDI was associated with increased mortality; pooled HR was 1.34 (95% CI: 1.24 to 1.44, p<0.001; Q p=0.027, I2=52.1%). Exclusion of one potential outlying study reduced heterogeneity with minimal impact on pooled HR (1.33 (95% CI: 1.25 to 1.42), p<0.001, Q p=0.087, I2=42.0%). The 11 remaining studies, although they could not be aggregated because of their varying patient populations and analyses, consistently demonstrated that greater SDI was associated with increased mortality.ConclusionsOrgan damage in SLE is consistently associated with increased mortality across studies from various countries. Modifying the disease course with effective therapies and steroid-sparing regimens may reduce organ damage, improve outcomes and decrease mortality for patients with SLE.

scholarly journals Variable presentations of systemic lupus erythematosus based on presence or absence of antidouble stranded DNA antibodies: A case series

2022 ◽  
Vol 13 (1) ◽  
pp. 175-179
Author(s):  
Somnath Maitra ◽  
Swapan Sarkar ◽  
Biswaroop Mukherjee ◽  
Suprotim Ghosh
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Clinical Features ◽  
Lupus Erythematosus ◽  
Renal Involvement ◽  
Case Series ◽  
Systemic Lupus ◽  
American College Of Rheumatology ◽  
European League Against Rheumatism ◽  
Dna Antibodies ◽  
European League

Systemic lupus erythematosus (SLE) presents with diverse clinical features causing diagnostic challenges. Apart from the clinical features, autoantibodies are important for diagnosis along with certain laboratory parameters. Diagnosis is made with the European League against Rheumatism/American College of Rheumatology 2019 Criteria. The case series presented here signifies the correlation between anti ds DNA positivity and its association with poor prognosis and renal disease, whereas antidouble stranded DNA (anti-dsDNA) negativity may lead to lack of renal involvement and may be associated with polyserositis. The importance lies in the fact that these patients with anti-dsDNA negativity should be followed up for assessing conversion to positivity of anti-dsDNA, thus altering the prognosis and leading to renal involvement. Moreover, anti-SSA positive SLE patients must be followed up for possible development of sicca symptoms.

scholarly journals Validity test of anti-c1q serum as diagnostic marker for lupus nephritis

2018 ◽  
Vol 8 (1) ◽  
Author(s):  
M Enrica ◽  
A Tjandrawati ◽  
S Rachmayati ◽  
Laniyati Hamijoyo
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Nephritis ◽  
Lupus Erythematosus ◽  
Predictive Value ◽  
Renal Involvement ◽  
Enzyme Linked Immunosorbent Assay ◽  
Specific Marker ◽  
Systemic Lupus ◽  
Urine Protein ◽  
American College Of Rheumatology

Background: Lupus nephritis is defined as renal involvement in systemic lupus erythematosus (SLE) patients and the most important cause of morbidity and mortality. The diagnostic criteria that used to diagnose lupus nephritis are 1997 American Collegeof Rheumatology is 24 hours urine protein ≥500 mg and/or cellular cast, but significant renal damage can occur without proteinuria or cellular cast. Anti-C1q is an autoantibody that is produced by a chronic alteration of C1q collagen domain. Anti-C1q is a new specific marker for renal marker.Objective: To determine the validity of anti-C1q serum by using 1997 American College of Rheumatology criteria as a gold standard. Methods: This is a cross sectional study, conducted in October to December 2014 at Hasan Sadikin Hospital Bandung. The subjects had systemic lupus erythematosus with and without renal involvement, based on 1997 American College of Rheumatology criteria for SLE.Results: There were 65 subjects included in this study, 64 subjects were female and 1 subject was male. The age average was 32 (SD 11.7) years old. As many as 66.2% subjects had been diagnosed with lupus erythematosus systemic at least 3 years. Twenty four hours urine protein was measured using spectrophotometry, urine sediment was examinedfor cellular cast, and anti-C1q serum was measured using micro enzyme linked immunosorbent assay. Based on American College of Rheumatology criteria, 34 subjects were classified as lupus nephritis group while 31 subjects were classified as non-lupus nephritis group. The area under the curve of anti-C1q was 0.610. The cut-off value used in this study was 10.43 U/ml. The sensitivity, specificity, positive predictive value,negative predictive value and accuracy of anti-C1q assay were 41.18%, 77.42%, 66.67%, 54.55% and 58.46% respectively.Conclusion: Anti-C1q assay, based on this study, hasa low sensitivity and medium specificity to detect lupusnephritis

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