Lupus nephritis (LN) may lead to end-stage kidney disease (ESKD) in 22% of patients over 15 years with the risk being particularly higher in diffuse proliferative forms. The rate of kidney function decline varies. However, a catastrophic course leading to ESKD within a few years from onset is uncommon. The aim of the present study was to assess the factors associated with rapid progression to ESKD in LN patients.Patients from the Toronto Lupus Clinic with biopsy-proven LN at presentation and eGFR≥60ml/min/1.73m2 who developed ESKD within three years were retrieved. Pathology reports were reviewed with particular emphasis on distinct histopathologic features. Demographic, clinical, laboratory and therapeutic variables were also analyzed.Ten patients (1.8% of the total LN population) developed ESKD within three years of diagnosis. Their mean age was 34.2±7.3 years, mean time to ESKD 19.2±12.4 months, initial eGFR=90.2±24.9ml/min/1.73m2, proteinuria 2.7±1.04 grams/24h. The rate of kidney function decline was more than 43ml/min/1.73m2/year (median). One patient had LN class III, five had LN class IV, two membranous LN (class V) and another two had mixed IV/V. Moreover, two patients had extensive thrombotic microangiopathy, one collapsing glomerulonephritis and one concomitant anti-glomerular basement membrane (anti-GBM) nephropathy. Four patients showed no unusual kidney pathology; all of them had severe non-compliance (discontinued all medications to follow alternative treatment).Catastrophic progression to ESKD is uncommon in LN. The major associated factors are poor compliance and distinct histopathologic features such as thrombotic microangiopathy, collapsing glomerulopathy and concomitant anti-GBM nephropathy.