To determine the clinical characteristics and long-term prognosis of 11 Japanese systemic lupus erythematosus (SLE) patients who were starting dialysis, we studied the clinicopathological findings before dialysis and during the clinical course after dialysis. Patients were divided into three groups: Group A consisting of temporary dialysis patients (n = 5); Group B consisting of early-death patient (n = 1); and Group C consisting of maintenance dialysis patients (n = 6). At the start of dialysis, the progression pattern of renal failure in all Group A patients was either acute exacerbation of chronic renal failure (CRF) or rapidly progressive glomerulonephritis (RPGN). The pattern in Group C was mostly CRF (83%). Three patients from Group A and three from Group C were still alive at the latest follow-up (mean follow-up period was 11.0 years, ranging from 1.8 to 16.2 years) and they showed no signs of clinical or serological activity. Infection was the cause of death in 3 of the 5 patients who died. We suggest that dialysis can be discontinued in patients with SLE who receive dialysis for acute exacerbation of CRF or RPGN. After the initiation of dialysis, patients tend to show diminished SLE activity. Infection is the major contributor to the poor prognosis of SLE patients receiving dialysis.