Clinical and laboratory profile, disease activity and outcome of childhood systemic lupus erythematosus - A retrospective study in a tertiary care hospital in Assam

Abstract

Background: Systemic lupus erythematosus in children (cSLE) represents 10-20% of all cases of SLE. However, this is still underreported from Assam. In this study, we report our experience with 25 patients of cSLE over the last 3.5 years. Materials and methods: This retrospective study was carried out at the Department of Pediatrics, Gauhati Medical College and Hospital, Guwahati, Assam. Data of patients with cSLE in the records of pediatric rheumatology and immunodeficiency clinic from April 2018 to October 2021 were collected and analysed. Results: In our cohort, the median age at presentation was 10 years (range, 4.6-14 years). The female-to-male ratio was 7:1. Hematological involvement, 21 (84%) was the most common manifestation followed by cutaneous, 19 (76%), and renal involvement, 17 (68%). Five patients also had associated HbE hemoglobinopathy. We had performed renal biopsy in 12/17 patients. Class IV lupus nephritis was the most common type in our patients. Twenty-four out of 25 patients had active disease at the time of the first presentation. There were four deaths in our cohort including two deaths that occurred due to congestive cardiac failure in patients with suspected myocarditis. Seventeen patients in our cohort had completed a median of 10 months (range, 2 – 30 months) follow-up and 6/9 biopsy-proven proliferative nephritis were in remission after a median of 9.5 months (range, 2-27 months) follow-up. Conclusion: Hematological involvement was the commonest manifestation in cSLE at our center. The majority of patients with proliferative LN were in remission with standard treatment.

Keywords

• hemoglobin e
• lupus nephritis
• myocarditis
• vasculitis