Lupus Nephritis in University of Port Harcourt Teaching Hospital, Port Harcourt, Nigeria

Abstract

Background: Paediatric-onset Systemic Lupus Erythromatosus (pSLE) is a severe autoimmune disease with multisystem involvement characterized by immune dysregulation and malfunctioning. Lupus Nephritis occurs in 60% to 80% of all pSLE patients, and increases the morbidity and mortality associated with this condition.

Methodology: This was a single center review of children managed for Lupus Nephritis in the Paediatric Nephrology unit of the University of Port Harcourt Teaching Hospital from June2017 to February 2020. Diagnosis of SLE was made using the EULAR/ACR scoring system and staging of Lupus Nephritis was based on the International Society of Nephrology and the Renal Pathology Society (ISN/RPS) 2018 Revised Grading of Lupus Nephritis.

Results:  There were seven cases of SLE with Lupus Nephritis managed at the centre during the period. These included five females (71.4%) and two males (28.6%), giving a female to male ratio of 2.5: 1. The ages of the patients ranged from 4.5years to 17years, with a median age of 12years. Renal manifestations included generalized body swelling in 100%, hypertension in 71.4% and haematuria in 85.7%. Their ISN/RPS grades were grades IIIA, IIIC, and IVA Extra renal manifestations were fever in 85.7%, joint pain/swelling in 71.4%), haematological manifestations with thrombocytopenia in 71.4% and severe anaemia in 57.1%, skin involvements in 42.9%, and a case of neuropsychiatric involvement. Treatment received included blood transfusion, steroid therapy, Mycophenolatemofetil, antihypertensives, diuretics/antihypertensives, and haemodialysis. Outcome was poor, with a mortality rate of 4 (57.1%).

Conclusion: Lupus nephritis is a severe disease and major cause of morbidity and mortality in children.