Background About one fifth of SLE patients have onset before the age of 18 years. Long term outcome of these patients is difficult to assess as they are partly managed in pediatrics and then transition to adult services. Our clinic provides care to both pediatric and adult SLE patients. Further data from developing countries is scant
Objectives Thus we decided to study the outcome of our cohort of juvenile onset SLE patients.
Methods Medical records of patients attending the SLE clinic for the last 25 years were scanned and patients with onset of disease before age of 18 years (JSLE) were identified. In them demographic data, clinical and laboratory features, medications used, outcome and complications was retrieved. Overall outcome as well as actuarial survival was calculated
Results There were 205 patients with JSLE among a total of 1015 SLE patients. Their median age at onset was 14 years (1-18 years) and mean duration of illness prior to presentation at this tertiary care hospital was 1 year (0.2-20 years). There were 292 girls and 13boys. Fever, alopecia, arthritis and renal involvement was present in more than two third of patients. One third had CNS disease with seizures being the commonest presentation. Among 130 patients with renal involvement kidney biopsy was done in Class I in 1, Class II in 13, class III in 24, class IV in 41 and class V in 12.
Median follow up was 4 years (0-24 years). Flares were seen in nearly half the patients with 66 patients having 1 flare (41being major flares), 24 patients having 2 flares (28 being major flares), 9 patients having 3 flares (16 being major), 4 having 4 flares (9 being major) and 1 patient having 6 flares (all major). Acturial 5 year survival was 95% and 10 year survival was 89%. 55 patients had an infective episode during the follow up with almost half requiring hospitalization. Eleven patients died (5 during first admission due to uncontrolled disease activity, 2 with ESRD, 1 TB, 1 PAH with CAPS, 1 staph sepsis, 1 diabetic ketoacidosis).
Conclusions JSLE in a developing country like India has good outcome but has significant morbidity due to disease flares and infections
Disclosure of Interest None declared