It is not unusual that patients with systemic lupus erythematosus (SLE) progress to terminal renal failure and subsequently require renal replacement therapy. Previous studies have shown that clinical and/or serological remission in patients with SLE is common in those who develop end-stage renal disease (ESRD). On the other hand, the persistence of lupus activity among patients undergoing long-term dialysis is not rare, either. The aim of this study is to define, by means of a systematic review, the course of SLE activity in patients who developed ESRD. Data were obtained through searches for articles in the MEDLINE (1966 to 2011), SCielo, and LILACS databases, using the following keywords: "chronic renal failure", "systemic lupus erythematosus", "end-stage renal disease", "lupus activity", "disease activity", "lupus flare", "hemodialysis", and "renal replacement therapy" and their corresponding translations in Portuguese. Twenty-four articles were found which evaluated the degree of lupus activity in patients with ESRD. Fifteen of these studies spoke of a substantial reduction of clinical and/or serological activity after the development of ESRD, while nine articles found that the amount of clinical and/or serological activity was similar to that of the phase prior to terminal renal failure, or it occurred in at least 50% of the patients studied. Although the majority of studies showed that lupus flares tend to decrease in frequency in patients who develop ESRD, in this scenario, one should be prepared to correctly diagnose a recurrence of the disease, as well as to perform appropriate therapy.