Chronic active disease pattern predicts early damage in juvenile systemic lupus erythematosus

Sato, Juliana de Oliveira; Corrente, José Eduardo; Saad‐Magalhães, Claudia

doi:10.1177/0961203315599449

Cited by 13 publications

(12 citation statements)

References 29 publications

(57 reference statements)

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“…Persistent active disease itself has been reported to predict early damage in children with lupus. 35 Although the use of corticosteroids has helped improve survival in lupus, the need for prolonged moderate to high doses of corticosteroids especially in children with HDA has resulted in significant cumulative adverse effects contributing to overall long-term damage. Certainly, the general observation worldwide is that these children with lupus survive longer but at the expense of more disease damage and many are corticosteroid related.…”

Section: Discussionmentioning

confidence: 99%

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Lim

Chan

Tang

2020

Lupus

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Background Paediatric systemic lupus erythematosus is a rare autoimmune disease with a wide spectrum of clinical presentation in different populations. We present a cohort of paediatric systemic lupus erythematosus in Malaysia where the disease features and outcomes are still largely unknown. Methods A retrospective review of all paediatric systemic lupus erythematosus patients with at least 6 months follow-up at Selayang Hospital from 2004 to 2016. Epidemiological, clinical and outcome data were collected and analysed. Results A total of 141 paediatric systemic lupus erythematosus patients, 87.9% females, were followed up for a median 6.3 years (interquartile range 3.6–9.0). The median age at diagnosis was 10.8 years (interquartile range 9.0–12.0 years), positive family history of systemic lupus erythematosus was present in 12.1% and the majority (61.7%) were of Malay ethnicity. Common presentations included fever (87.2%), vasculitic rash (72.3%) and lethargy (69.5%). At diagnosis, leukopenia (51.1%), thrombocytopenia (41.8%) and cutaneous lupus (56%) predominate with significant renal involvement (39.7%). Renal (45.4%), liver (26%) and the central nervous system (17%) were important major organs involved during the course of the disease. At diagnosis, almost all (99.3%) patients had high disease activity (mean Systemic Lupus Erythematosus Disease Activity Index score 20.1 ± 9.6). The majority (62.4%) achieved remission or low disease activity after 6 months, maintained over the next 10 years. Damage occurred early (39.1% at 1 year) and increased with time. Ocular damage was the most common side effect (29%) and was predominantly corticosteroid related (93%). Growth retardation was significant (38.2%) with no gonadal failure or secondary malignancies. End-stage renal disease occurred in 3.1% patients whereas 53.1% had sustained renal remission. Overall mortality was 1.4%. Conclusion Despite high disease activity at diagnosis, the majority had good sustained response to treatment with low overall mortality. However, there was progressive accrual of organ damage, highlighting the need for further research and refinements into therapies for paediatric systemic lupus erythematosus.

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Section: Discussionmentioning

confidence: 99%

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Lim

Chan

Tang

2020

Lupus

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“…Scoring indices that track treatable disease activity and nonreversible damage have been developed for lupus (57). Single center studies from Canada and Brazil have validated the utility of these activity and damage indices in cSLE patients (58,59), but modifications of the scales developed for adults with SLE have improved overall accuracy (60). Outcomes research in cSLE has also evolved to include health-related quality of life (HRQOL).…”

Section: Tools To Monitor Sle and Advances In Chronic Disease Managementmentioning

confidence: 99%

Advances in the care of children with lupus nephritis

2016

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The care of children with lupus nephritis (LN) has changed dramatically over the past 50 y. The majority of patients with childhood-onset systemic lupus erythematosus (cSLE) develop LN. In the 1960's, prognosis in children was worse than in adults; therapies were limited and toxic. Nearly half of cases resulted in death within 2 y. Since this time, several diagnostic recommendations and disease-specific indices have been developed to assist physicians caring for patients with LN. Pediatric researchers are validating and adapting these indices and guidelines for the treatment of LN in cSLE. Classification systems, activity, and chronicity indices for kidney biopsy have been validated in pediatric cohorts in several countries. Implementation of contemporary immunosuppressive agents has reduced treatment toxicity and improved outcomes. Biomarkers sensitive to LN in children have been identified in the kidney, urine, and blood. Multi-institutional collaborative networks have formed to address the challenges of pediatric LN research. Considerable variation in evaluation and treatment has been addressed for proliferative forms of LN by development of consensus treatment practices. Patient survival at 5 y is now 95-97% and renal survival exceeds 90%. Moreover, international consensus exists for quality indicators for cSLE that consider the unique aspects of chronic disease in childhood.

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“…The relationship between disease activity and organ damage showed that, independent from the mean SLEDAI score at disease onset, patients with chronically active disease were at significantly higher risk of developing organ damage; studies by Brunner and Sato showed similar conclusions, confirming that cumulative disease activity is a predictor for organ damage ( 35 , 36 ). To our knowledge, the present study is the longest follow-up study investigating the relationship between disease activity and organ damage in pediatric-onset disease.…”

Section: Discussionmentioning

confidence: 82%

Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood

et al. 2018

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Background: Pediatric systemic lupus erythematosus (pSLE) is a rare condition, representing approximately 10% of SLE cases. The aim of this study was to identify variables to improve the diagnostic awareness and management of pSLE patients.Methods: This retrospective study included 25 patients diagnosed with pSLE and followed at the University of Pisa. We collected data about clinical profile at disease onset and during a long-term follow-up, including disease activity, organ damage development, and treatments received.Results: The mean patient age at disease onset was 14.6 ± 1.6 years, and the mean follow-up period was 14.17 ± 8.04 years. The most common initial manifestations were arthritis, malar rash, and cytopenias. The median time to diagnosis since the first symptoms was 6 months, and was significantly longer in patients with hematological onset (54 months). During follow-up, the number of patients with renal involvement showed a significant increase, from 36% at diagnosis to 72.2% after 10 years of disease evolution. Patients who developed chronic organ damage maintained a higher time-averaged disease activity during follow-up and received a significantly higher dose of corticosteroids.Conclusion: Patients with immune cytopenia represent a group deserving strict clinical follow-up for the risk of evolution to SLE. Intense surveillance of renal function, early treatment and steroid-sparing strategies should be strongly considered in the management of pSLE patients.

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Chronic active disease pattern predicts early damage in juvenile systemic lupus erythematosus

Abstract: Damage was observed in nearly two-thirds of patients, and occurred earlier in the chronic active pattern. Disease duration, thrombocytopenia and alopecia at onset predicted damage.

Cited by 13 publications

References 29 publications

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Clinical features, disease activity and outcomes of Malaysian children with paediatric systemic lupus erythematosus: A cohort from a tertiary centre

Advances in the care of children with lupus nephritis

Pediatric Systemic Lupus Erythematosus: Learning From Longer Follow Up to Adulthood

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