Abstract: Objective The incidence, presentation and outcomes of lupus nephritis (LN) varies with geography, ethnicity, socioeconomic status, and gender. There is relatively few data on LN in the non-Caucasian populations in Australia. This study describes the clinical presentation, histological features, natural history, and outcomes of a historical cohort of Aboriginal and Torres Strait Islanders people in Far North Queensland with biopsy-proven LN. Design Retrospective observational study. Setting Cairns and Hinterland Hospital and Health Service, Queensland, Australia. Participants Aboriginal and Torres Strait Islander patients with biopsy-proven LN treated between 1990 and 2013. Main outcome measures Renal replacement therapy and overall patient survival. Results Aboriginal and Torres Strait Islander people represented a substantial proportion (n=16/40, 40%) of all patients diagnosed with LN during the observation period. The frequency of nephrotic range proteinuria (n=11/14, 78.5%), eGFR <60 mL/min/1.73m2 (n=6/14, 42.8%) and proliferative LN (n=13/16, 81.25%) was high at the time of presentation. Despite use of multiple immunosuppressive agents, the overall rate of remission was poor (n=4/14, 28.5%), and incidence of ESKD (n=4/14, 28.5%) and death (n=5/16, 31.25%) was high. Conclusion The clinical presentation of LN in Aboriginal and Torres Strait Islanders in Far North Queensland is severe and the response to standard immunosuppressive therapy is unsatisfactory. Larger prospective multi-centre studies are required to better understand ethnic disparities in prognosis and response to immunosuppressive therapy in this specific population. This article is protected by copyright. All rights reserved.