Bonnie Abujam1, Anju Gupta1, Deepti Suri1, Amit Rawat1, Surjit Singh2. 1. Paediatric Allergy Immunology Unit, Advanced Pediatric Centre, Post Graduate Institute of Medical Sciences, Chandigarh, India. 2. Paediatric Allergy Immunology Unit, Advanced Pediatric Centre, Post Graduate Institute of Medical Sciences, Chandigarh, India. surjitsinghpgi@rediffmail.com.
Abstract
OBJECTIVES: Systemic lupus erythematosus has been shown to be associated with worse survival in developing countries in adults and children. However, there are limited data on causes of death and time-trends of mortality in childhood lupus from developing countries. The aim of this study was to determine any changes in occurrence (time-trends) and aetiology of mortality over 23 years at our centre and to determine the risk factors associated with mortality. METHODS: This retrospective study included patients with childhood onset lupus (fulfilling ACR 1997) who were diagnosed at the Paediatric centre of a North-Indian federally funded university hospital from 1991 to 2013 and were below 14 years of age at presentation (age cut-off used to register patients in the Paediatric centre). Patients were divided into three cohorts based on their year of presentation, i.e. 1991-1998, 1999-2006 and 2007-2013 to evaluate differences in survival over time at our centre. Survival was estimated by Kaplan-Meier analysis and predictors of mortality were analysed by Cox-regression. A worst-case-scenario was also calculated by assuming patients lost to follow-up as not survived. RESULTS: This study included 122 children (F:M=3:1) with childhood onset SLE having a mean age of onset of lupus and presentation to our centre being 9.2±2.7 years and 10.5±2.7 years. During a mean follow-up of 4.8±4.5 (range 0-20) years, 24 children (20%) died. Survival rates at 1, 5 and 10 years was 88.3, 77.5 and 70.9%. In the worst case scenario, the survival was 78.3, 59.5 and 38.8% respectively. Only serum creatinine was significantly associated with time-to-event (death) (Hazard ratio 2.9; 95% CI: 1.3-6.8; p=0.008). There was a significant improvement in survival in patients that presented between 1999-2006 compared to patients who presented in 1992-1998 (p=0.03). Among the patients who died, 11 died at the time of first admission, whereas 13 died later. Major cause of mortality in former was infection and active disease, and in the latter was end-stage renal disease. CONCLUSIONS: In this single-centre study, childhood onset lupus was associated with a mortality of 20%, with the major predictor of mortality being serum creatinine at presentation. A comparison of different cohorts of patients found an improvement in survival over time at our centre.
OBJECTIVES: Systemic lupus erythematosus has been shown to be associated with worse survival in developing countries in adults and children. However, there are limited data on causes of death and time-trends of mortality in childhood lupus from developing countries. The aim of this study was to determine any changes in occurrence (time-trends) and aetiology of mortality over 23 years at our centre and to determine the risk factors associated with mortality. METHODS: This retrospective study included patients with childhood onset lupus (fulfilling ACR 1997) who were diagnosed at the Paediatric centre of a North-Indian federally funded university hospital from 1991 to 2013 and were below 14 years of age at presentation (age cut-off used to register patients in the Paediatric centre). Patients were divided into three cohorts based on their year of presentation, i.e. 1991-1998, 1999-2006 and 2007-2013 to evaluate differences in survival over time at our centre. Survival was estimated by Kaplan-Meier analysis and predictors of mortality were analysed by Cox-regression. A worst-case-scenario was also calculated by assuming patients lost to follow-up as not survived. RESULTS: This study included 122 children (F:M=3:1) with childhood onset SLE having a mean age of onset of lupus and presentation to our centre being 9.2±2.7 years and 10.5±2.7 years. During a mean follow-up of 4.8±4.5 (range 0-20) years, 24 children (20%) died. Survival rates at 1, 5 and 10 years was 88.3, 77.5 and 70.9%. In the worst case scenario, the survival was 78.3, 59.5 and 38.8% respectively. Only serum creatinine was significantly associated with time-to-event (death) (Hazard ratio 2.9; 95% CI: 1.3-6.8; p=0.008). There was a significant improvement in survival in patients that presented between 1999-2006 compared to patients who presented in 1992-1998 (p=0.03). Among the patients who died, 11 died at the time of first admission, whereas 13 died later. Major cause of mortality in former was infection and active disease, and in the latter was end-stage renal disease. CONCLUSIONS: In this single-centre study, childhood onset lupus was associated with a mortality of 20%, with the major predictor of mortality being serum creatinine at presentation. A comparison of different cohorts of patients found an improvement in survival over time at our centre.
Authors: Laura B Lewandowski; Melissa H Watt; Laura E Schanberg; Nathan M Thielman; Christiaan Scott Journal: Pediatr Rheumatol Online J Date: 2017-02-23 Impact factor: 3.054