Clinico-immunological profile and outcome of childhood systemic lupus erythematosus

Introduction: Paediatric lupus is an autoimmune disorder most commonly affecting adolescent females. Various studies regarding paediatric lupus have been reported across the world. Objective: To study the clinical and immunological features of SLE along with treatment modalities and the response at the end of one year follow up. Relationship between the types of autoantibodies and probability of systemic involvement is also assessed. Method: A prospective and retrospective observational study was carried out in the Paediatric Department of a tertiary care hospital from January 2010 to July 2016.  Subjects included children from 1 month to 18 years of age fulfilling the 1997 American College of Rheumatology criteria for systemic lupus erythematosus (SLE). Results: Study population included 44 children fulfilling the criteria. Among them 10 were in the prospective group and 34 in the retrospective group. Female: male ratio was 4.5:1. Median age at diagnosis was 13.2 years (interquartile range 11.2-14.6). Presenting features were constitutional in 86.4%, musculoskeletal in 72.7%, renal in 65.9%, haematological in 63.6%, muco-cutaneous in 61.3%, central nervous system in 36.3% and serositis in 25% children. Anaemia was the commonest haematological abnormality and was found in 75% of children. Hypocomplementaemia was seen in 100% of children. All subjects were positive for antinuclear antibodies. Anti-double stranded DNA (77.2%) was the most commonly observed autoantibody profile followed by anti-ribosomal P protein (47.7%) and anti-ribonucleoprotein (43.1%). During follow up of 36 children, 19 (52.7%) children attained complete remission, 8 (22.3%) went into partial remission and 9 (25%) had persisting active disease. During the study period 13 of 44 children (29.5%) succumbed either to the active disease process or to complication of SLE. Conclusions: The clinical presentation and course of progression of the disease varies depending on the age of onset and the organ system involved. Low complement levels indicate activation of the disease especially lupus nephritis. Sri Lanka Journal of Child of Child Health, 2019; 48(3): 201-207