Lupus nephritis in children at Chris Hani Baragwanath Academic Hospital, an observational study from January 2000 to December 2019

Abstract

Lupus nephritis in children at Chris Hani Baragwanath Academic Hospital, an observational study from January 2000 to December 2019. Ritshidze Mulaudzi 1,2Anees Laher 1,2, Pulane Mosiane 3,4, Karen L Petersen 1,2. Affiliations: 1. Faculty of Health Sciences, University of the Witwatersrand 2. Department of Paediatrics and Child Health, Chris Hani Baragwanath Academic Hospital 3. Department of Pathology, National Health Laboratory Service 4. School of Pathology, University of the Witwatersrand This is a retrospective study conducted at the Paediatric Renal Unit at Chris Hani Baragwanath Academic Hospital on patients under the age of eighteen years who had biopsy-confirmed lupus nephritis between January 2000 and December 2019. This study reports the outcomes and complications of childhood Lupus Nephritis in a predominantly Black population in Low and Middle-Income Countries, with multiple indicators of severity. vii Background: Lupus nephritis (LN) is a severe manifestation of systemic lupus erythematosus resulting in worse outcomes than those without kidney involvement. Methods: We conducted a retrospective study reviewing records of children with biopsy-proven lupus nephritis, treated at the Paediatric Renal Unit of Chris Hani Baragwanath Academic Hospital from January 2000 to December 2019. Patients were identified using the renal biopsy register. Lupus nephritis was classified using the International Society of Nephrology/Renal Pathology Society (ISN/RPS) classification system. Repeated biopsies from a single patient and those with insufficient records or no histology confirmation were excluded. Risk factors (initial clinical or laboratory manifestations, histo-pathological patterns and treatment modalities) for advanced chronic kidney disease were assessed. Results: A total of 833 percutaneous kidney biopsies were performed during the study period. Fifty-two (6.2%) were identified to have lupus nephritis, and 44 cases were analysed (seven had incomplete records and one child had inconclusive histology). The mean age at presentation was 11.8 (IQR, 11.3 to 12.1) years. Females accounted for the majority of cases at 33 (75%). Common renal manifestations at presentation were nephrotic range proteinuria seen in 26 (59.1%) patients, and elevated creatinine in seven (15.9%) patients. Common extrarenal manifestations viii included dermatological in 31 (70.4%), musculoskeletal in 14 (31.8%), and neuro-lupus in 12 (27.3%). Anti-dsDNA antibody was positive in 36 (81%); antiphospholipid antibodies in 11 (n=30, 36.6%). The mean Systemic Lupus Erythematosus Disease Activity Index score was 14.6±1.2. Macrophage activation syndrome was diagnosed in seven (15.9%). Proliferative LN (class III, IV, and mixed) were seen in 28 (63.6%). Remission was achieved in 25 (56.8%). Two (4.5%) patients died and three (6.8%) patients developed advanced kidney disease (CKD 3,4 and 5). The mean (±SD) duration of follow-up was 52.5±6.9 months. Conclusions: Lupus nephritis presents commonly with proteinuria in this study. Class III LN is the dominant pathology. Screening for antiphospholipid and macrophage activation syndromes is recommended. Young age is associated with severe disease.