THU0510 Renal Outcomes in a Juvenile-Onset Systemic Lupus Erythematosus Cohort – Experience of a Tertiary Center
Authors: M. Rodrigues, F. Aguiar, A. Caldas Afonso, I. Brito
Journal: Annals of the Rheumatic Diseases (2015)

<h3>Background</h3> Juvenile systemic lupus erythematosus (jSLE) is rare but associated with a worse prognosis than adult-onset disease. This has been attributed to higher risk of renal involvement, a tendency to have more active disease over time, receive more intensive immunosuppressive treatment and accrue more damage over a longer duration of disease. <h3>Objectives</h3> To describe the pattern of disease expression with special focus on renal outcomes in a cohort of patients with jSLE in a tertiary hospital. <h3>Methods</h3> Retrospective analysis of medical records in a group of jSLE patients. Statistical analysis was done using Excel 14.4.5 and SPSS 20.0. <h3>Results</h3> 25 patients were included, with disease onset between 6 and 17 years of age (mean 12.5), and mean disease duration of 7.8 years (1-28). 23 (92%) patients were females. Current mean age is 20.4±5.9 years (13–37). The most common disease manifestations were mucocutaneous (84%), musculoskeletal (68%) and hematological abnormalities (68%). Lupus nephritis was diagnosed in 11 (44%) patients, all confirmed with renal biopsy. 9 patients had class IV (2003 ISN/RPS), 1 class V and 1 class V + II nephritis. According to the EULAR/ERA-EDTA 2012 renal response criteria, after 6 months, 7 (64%) had complete response to treatment, 1 (9%) partial response and 3 (27%) remained active. After 12 months, 8 (73%) had complete response and 3 (27%) remained active. The 3 patients with renal refractory disease at 6 months were diagnosed before 2003, had class IV nephritis and received monthly IV cyclophosphamide (CY) and pulse methylprednisolone (MP). 3 patients (2 class IV and 1 class II+V nephritis) received mycophenolate mofetil (MMF), plus steroids, as induction and maintenance therapy and all achieved complete remission, with an average follow-up of 1.3 years. During follow-up of the 6 and/or 12-month responsive patients, 1 flared after 15 months, 1 after 10 years and 1 after 6 years (all class IV nephritis), which were treated with add-on rituximab (after failing CY and/or MMF); 2 had complete remission after 6 months and 1 had partial remission. All patients with lupus nephritis received corticosteroids, 8 (72%) pulse IV MP, 10 (90%) MMF, 8 (72%) IV CY, 7 (64%) azathioprine, 6 (54%) hydroxychloroquine and 9 (82%) angiotensin-converting enzyme inhibitors. Only 1 patient has chronic kidney disease (Stage II - Glomerular Filtration Rate 76 mL/min/1.73m<sup>2</sup>) who is currently 37 years old, never achieved complete renal remission and is morbidly obese. <h3>Conclusions</h3> Our results are in agreement with the literature, with a high incidence of lupus nephritis in juvenile-onset patients. Despite a limited follow-up, induction treatment followed by maintenance with MMF in our cohort achieved 100% complete response rates. Rituximab was effective as rescue add-on therapy in refractory disease. Renal and vital outcomes were good, with no patients with end-stage renal disease or deaths. However, we need long-term follow-up to properly evaluate how the degree of renal involvement affects the mortality and morbidity of patients in the cohort and how newer treatment options will fare in the years to come. <h3>Disclosure of Interest</h3> None declared