A-15-year experience of paediatric systemic lupus erythematosus (pSLE) in Hospital Universiti Sains Malaysia
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Date
2014
Authors
Allamani Alin, Jamil Mohammed
Journal Title
Journal ISSN
Volume Title
Publisher
Universiti Sains Malaysia
Abstract
Introduction: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder, relatively rare and under-reported in paediatric age group, affecting females in the majority of cases. The disease manifestations and clinical outcomes vary depending on which system being involved and treatment response. Its etiology is complex and involves an interaction between genetic, hormonal and environmental factors. Diagnosis is made clinically based on certain clinical and laboratory criteria made by American College of Rheumatology (ACR).
Objectives: The aims of the study were to describe the demography, clinical and laboratory manifestations, and outcomes of children with SLE and to determine the risk factors associated with the development of renal failure and death among SLE children admitted to Hospital USM in the period between 1996 and 2010.
Patients and Methods: In this cross sectional study we retrospectively reviewed the folders of 51 children over a period of 15 years; all patients were below 18 years of age and fulfilled the SLE criteria outlined by American College of Rheumatology.
Results: The median age was 12 years with female predominance, male to female ratio was 1:10. Majority of our patients have positive family history and the commonest clinical manifestation was involvement of haematological and renal systems. One quarter of the cohort developed lupus nephritis some requiring acute dialysis but none necessitating long term dialysis or progressed to end stage renal disease (ESRD). Death complication was observed in almost 30% of the cases, majority due to sepsis.
Conclusion: The sociodemography, clinical profile, laboratory findings and outcome of childhood SLE in Kelantan are not much different from other studies done abroad. Lupus nephritis was found to be a risk factor for development of renal failure in this group of patients whereas infection was the leading cause of death; however, the other studied factors associated with these outcomes among our SLE children need further larger studies to be concluded.
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Keywords
Systemic lupus erythematosus (SLE)