Kidney involvement in systemic lupus erythematosus (SLE), known as lupus nephritis (LN), may be found in up to 60 % of patients [1]. Kidney manifestations usually present concomitantly with an SLE diagnosis or shortly thereafter and may vary from subtle findings in the urine (e.g., hematuria, low-grade proteinuria) to a rapidly progressive nephritic syndrome [2]. Despite advances in the management of SLE, and chronic kidney disease more generally, 10–30 % of patients with LN progress to kidney failure within 10 years of diagnosis [3]. Importantly, LN is also associated with increased mortality, with standardized mortality ratios of 2.2, 3.6, and 9.20 for patients with kidney involvement, kidney damage, or kidney failure, respectively; compared to patients with SLE and no renal involvement [4]. Morbidity secondary to the disease or its treatment is also increased in LN, with increased incidence of cardiovascular events, infections, and metabolic complications, among others [[5], [6], [7]]. Finally, LN is associated with impairment of health-related quality of life [8].

In the last few years, new medications have demonstrated benefit in short term outcomes of LN and two of them have already been approved by regulatory agencies to be used as initial therapy, in combination with existing standard of care. As new therapies emerge, the challenge for physicians caring for patients with LN will be to select the combination of therapies that will most likely benefit an individual patient, whilst minimizing the risks caused by immunosuppression. Furthermore, the emergence of new therapies also modifies how we interpret clinical findings, biomarkers, and histology during treatment follow-up. In this review, we provide an up-to-date summary of the diagnosis, management, and follow-up of patients with LN.