PAPER

The objective of this study was to clarify the long-term outcome in patients with lupus neph-ritis (LN) according to the International Society of Nephrology and Renal Pathology Society classification. This retrospective analysis comprised 186 Japanese patients given a diagnosis of LN by renal specimen with a mean observation period of 12 years. Primary end point was defined as death or end-stage renal disease, and standardized mortality ratios were calculated. Five patients presented with histopathological class I, 62 with II, 21 with III or IIIþV, 73 with IV or IVþV and 25 with V. Fourteen deaths occurred, corresponding to an overall standar-dized mortality ratio of 3.59 (95 % confidence interval 2.02–5.81, p< 0.0001). Kaplan-Meier analysis revealed a 10-year overall survival of 95.7%. Nephrotic proteinuria (3.5 g/day) at baseline was identified as an independent poor prognostic factor for overall survival in Cox regression analysis. Kaplan-Meier analysis revealed a 10-year renal survival as 94.3%. Male gender and nephrotic proteinuria at baseline were identified as independent poor prognostic factors for renal survival in Cox regression analysis. In conclusion, LN was associated with a 3.59-fold increase in mortality compared with the general population. Male gender and neph-rotic proteinuria were predictive for poor renal outcome. Lupus (2014) 0, 1–9. Key words: Nephritis; renal lupus; systemic lupus erythematosu