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Objective To summarize clinical and imaging features of posterior reversible leukoencephalopathy syndrome (PRES) caused by systemic lupus erythematosus (SLE) and nephrotic syndrome (NS). Methods A total of 53 patients clinically diagnosed with PRES from January 2013 to December 2017 were selected. The patients were divided into SLE group (31 patients) and NS group (22 patients) according to their primary diseases. Differences between the 2 groups in imaging features, affected sites and severity of lesions were compared. Results Incidence rates of epileptiform seizure (χ2 = 9.255, P = 0.002) and visual impairment (χ2 = 7.005, P = 0.008) of the NS group were higher than those in the SLE group. In the SLE group, bilateral periventricular areas (adjusted χ2 = 5.377, P = 0.020), basal ganglia(adjusted χ2 = 4.825, P = 0.028) and centrum semiovale (χ2 = 8.113, P = 0.004) were mainly involved. The differences of imaging type between the 2 groups were statistically significant (Fisher's exact probability: P =0.029). The proportion of parietal occipital lobe distribution in NS group was significantly higher than that in SLE group (Fisher's exact probability: P = 0.016). After treatment, PRES recovery in the SLE group was better and faster than that in NS group (t = 2.612, P = 0.013). Conclusions Common pathogens of PRES namely SLE and NS are different in certain degree of imaging features, and this will be significant to understand pathophysiological mechanism and clinical diagnosis of this disease. DOI：10.3969/j.issn.1672-6731.2019.09.015