BACKGROUND: Lupus nephritis (LN) is common feature of Systemic Lupus Erythematosus (SLE) and affects 50% of patients with SLE. Racial differences in incidence and prevalence have been well documented worldwide. In Australia, higher incidence and prevalence of SLE had been previously reported in Aboriginal and Torres Strait Australians compared to non-Indigenous Australians. AIM: to describe the differences in clinical features and lupus biomarkers between Aboriginal and Torres Strait Islander Australian and non-Indigenous Australian LN patients. METHODS: We retrospectively identified all consecutive biopsy-proven LN patients in our institution and compared the clinical features and lupus biomarkers between Aboriginal and Torres Strait Islander Australians and non-indigenous Australians. RESULTS: Of the thirty-three consecutive biopsy proven LN patients, twenty-six self-identified as of Aboriginal and Torres Strait Islander descent. The estimated incidence of lupus nephritis in Aboriginal and Torres Strait Islander Australian and non-Indigenous Australians were 5.08 and 0.47 per 100,000 patient years respectively. Neurological manifestations (23.08% vs 0%), haematological manifestations (46.50 % vs 16.67) and right heart catheter proven pulmonary arterial hypertension (23.08% vs 0%) were more frequently observed amongst Indigenous Australian patients compared to non-Indigenous Australian patients. The incidence of positive Extractable Nuclear Antigen (ENA) was also higher among Indigenous Australian patients (84.62% vs 57.14%). CONCLUSION: Our study further supports the observation that lupus in Aboriginal and Torres Strait Islander Australians were of a 'distinct phenotype' compared to non-Indigenous Australians. Future research should be aimed at delineating the reason for this observed difference. This article is protected by copyright. All rights reserved.