Abstract

To determine the long-term outcome of Maori and Pacific Island children diagnosed with childhood onset lupus nephritis. A chart review was conducted of children diagnosed with biopsy proven lupus nephritis seen by the Starship Hospital and Kidz First paediatric rheumatology and/or Starship renal services between January 1992 and January 2018. Baseline and follow-up kidney histology, adherence and response to therapy including partial or full renal remission, refractory disease, end-stage kidney disease (ESKD) and mortality were determined. In a New Zealand cohort of 42 with childhood onset lupus nephritis, Maori and Pacific Island children were significantly more likely to develop class 4 lupus nephritis (RR (95% CI), 11.3 (3.84-49.9), p < 0.0001), demonstrate medication nonadherence (RR (95% CI) 12.4 (3.48-85.7), p < 0.0001) and experience end stage kidney disease (RR (95% CI) 15.7 (2.97-389.3), p = 0.0003) and mortality (RR (95% CI) 11.1 (1.91-280.1), p = 0.005) compared to non-Maori and Pacific Island children. In addition, Maori children with childhood onset lupus nephritis developed chronic histological changes significantly more rapidly than Pacific or Asian children (p = 0.038). Lupus nephritis is more common among Maori and Pacific Island children in New Zealand associated with a significant incidence of end stage kidney disease and mortality, with some Maori children developing rapid histologic disease progression.

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