Background Systemic lupus erythematosus is associated with poorer survival in developing countries in adults. However, there is limited data on survival and causes of death in childhood lupus
Objectives To identify cause, time-trends and predictors of mortality in North Indian children with SLE.
Methods This study included patients with childhood onset lupus who were diagnosed at a North Indian Federally funded university hospital from 1991 to 2014. Patients were included in this study if they fulfilled the ACR 1997 revised criteria for systemic lupus erythematosus and were below the age of 14 years on presentation. Clinical data was extracted from the medical records, to identify etiology in children who had died. To compare time trends of mortality, we divided patients into three cohorts on their year of presentation, i.e. 1991-1998, 1999-2006 and 2007-2013. Survival was estimated by Kaplan-meier analysis and compared using log-rank test. Univariate and multivariate analysis for predictors of mortality was done by cox-regression.
Results Among the 124 patients with childhood onset SLE who presented to our center, there were 24 deaths (20%). Eleven of these patients (46%) died at the initial visit or within 3 months of presentation. 1, 5 and 10 year survival was 88.3, 77.5 and 70.9% respectively. Common cause of death was presence of both infection and disease activity (42%). Among 11 patients with infection as a cause of death, sepsis, commonly polymicrobial or gram negative, occurred in 7, CNS tuberculosis in 2 and invasive fungal infection in 2. Disease activity related causes of death included active nephritis in 12, NPSLE in 3 and one case of myocarditis, diffuse alveolar hemorrhage and macrophage activation syndrome. The only baseline characteristic, which was a predictor of survival, was serum creatinine (hazard ratio 2.9 (95% CI 1.3 to 6.5)). There was a significant increase in survival from 1992-1998 to 1999-2006 (Figure 1). This was mainly contributed by the major decline in the proportion of patients who died at initial presentation from 25% to 8% in 1992-1998 and 1999-2006 respectively.
Conclusions This cohort of childhood lupus from a center in a developing country found an overall mortality in 20%, with almost half of these occuring at presentation. Presence of active disease and infection were the most common causes. However, there has been an improvement of survival with time.
Disclosure of Interest None declared