Posterior reversible encephalopathy syndrome (PRES) is an acute neurological syndrome. There are many reports of PRES occurring in the setting of rheumatic diseases. However, it remains uncertain whether rheumatic diseases are truly a risk factor for PRES, as the literature consists of case reports and small clinical series. Here, we evaluated the relationship between PRES and the rheumatic diseases, using a large population-based data set as the reference.
We conducted a medical records review of hospitalizations in the United States during 2016 with a diagnosis of PRES. Hospitalizations were selected from the National Inpatient Sample. International Classification of Diseases, 10th Revision, Clinical Modification codes were used to identify rheumatic diseases. A multivariate logistic regression analysis was used to calculate odds ratios (ORs) for the association of PRES and rheumatic diseases.
There were 3125 hospitalizations that had a principal billing diagnosis of PRES. Multivariate logistic regression revealed the multiple independent associations with PRES. The demographic and nonrheumatic associations included acute renal failure (OR, 1.52), chronic renal failure (OR, 12.1), female (OR, 2.28), hypertension (OR, 8.73), kidney transplant (OR, 1.97), and preeclampsia/eclampsia (OR, 11.45). Rheumatic associations with PRES included antineutrophil cytoplasmic antibody-associated vasculitis (OR, 9.31), psoriatic arthritis (OR, 4.61), systemic sclerosis (OR, 6.62), systemic lupus erythematosus (SLE) nephritis (OR, 7.53), and SLE without nephritis (OR, 2.38).
This analysis represents the largest sample to date to assess PRES hospitalizations. It confirms that several rheumatic diseases are associated with PRES, including antineutrophil cytoplasmic antibody-associated vasculitis, systemic sclerosis, SLE, and psoriatic arthritis. Acute and unexplained central nervous system symptoms in these patient populations should prompt consideration of PRES.

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