SAT0186 Ω MORTALITY IN CHILDREN WITH JUVENILE SYSTEMIC LUPUS ERYTHEMATOSUS IN AN ARGENTINE PEDIATRIC CENTRE.

Abstract

Background: Juvenile systemic lupus erythematosus (JSLE) is a complex multisystemic autoimmune disease, often causing irreversible damage, reduced quality of life and life expectancy. Data on causes of death and time trends in infant lupus mortality are limited. Objectives: The aim of this study was to determine mortality in a pedia-tric cohort. Methods: This retrospective study included patients with childhood onset lupus (fulfilling ACR 1997) who were diagnosed at the Paediatric centre in Santa Fe Argentine, Alassia Children Hospital from 1991 to 2018 and had 15 years of age at presentation. Results: This study includes 54 JSLE children (F:M=12, 5:1) with a mean age of onset lupus 12 years (range 4-15 years). During a mean follow-up in our centre of 3 years (range 12m-10 years), 12 patients (24%) was died; 5 during the follow-up in our centre and 7 in adult centre, 4 lost to follow-up. The average age of death was16 years (range 10-29 years) and the average since diagnose time was 17 months (range 3m-15 years). The principal system involved was renal (100%). 10 patients with diffuse proliferative lupus nephritis (WHO class IV), 2 membranous lupus nephritis (WHO class V), 5 patients (41.6%) with hematological disorders (2 pancytopenia, 2 leukopenia, 1 Macrophage Activation Syndrome). 3 patients had thrombosis. 5 patients (41.6%) with neurological involvement (4 psychosis, 1 depression). One patient with liver dysfunction. There one death related to renal biopsy. The principal cause of mortality was active disease and infection. Menin-gococcal meningitis, Klebsiella peritonitis, Pneumococcal cellulitis of the neck, peritonitis and 1 patient with disseminated tuberculosis. 11 patients (91, 6%) had received intravenous steroids as pulses and Cyclophosphamide pulse therapy, azathioprine or mycophenolate mofetil, hydroxychloroquine immunoglobulins. In some patients peritoned dialysis and ventilation. 8 patients (66%) with bad socioeconomic environment. Four patients (33%) with poor adherence to treatment. Conclusion: In this single-center study, childhood onset lupus was associated with a mortality of 24%. With higher frequency of aggressive renal disease and higher requirement for steroids and immunosuppressive drugs. Pediatric care should consider transition to adult's centers and ensure continuity of treatment.