Rheumatic Diseases Associated With Posterior Reversible Encephalopathy Syndrome

Abstract

ObjectivePosterior 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.MethodsWe 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.ResultsThere 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).ConclusionsThis 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.

A Young Patient with Microscopic Polyangiitis Requiring Hemodialysis with Complications of Repeated Episodes of Posterior Reversible Encephalopathy Syndrome Probably Due to Different Etiologies

“…A very recent analysis demonstrated a strong relationship between PRES and autoimmune diseases, including AAV, using a large population-based data set as the reference ( 12 ). Autoimmune disease is considered to cause PRES by mechanisms of both vasogenic edema and endothelial dysfunction ( 13 ).…”

The Association of Hypertension With Posterior Reversible Encephalopathy Syndrome in Systemic Lupus Erythematosus Patients: A Systematic Review

“…Antineutrophil cytoplasmic antibody-related vasculitis, psoriatic arthritis, systemic sclerosis, SLE with nephritis, and SLE without nephritis were among the rheumatic conditions linked with PRES (odds ratio (OR), 9.31, 4.61, 6.62, 7.53, and 2.38, respectively) [ 14 ].…”