The treatment is primarily supportive, consisting of treating seizures and hypertension and removing offending medications.
A detailed medication history is helpful to determine the triggering medication because the majority of reactions occur within two weeks of initiation, although in about 20% of cases, PRES from a medication can occur weeks or months later.6 Escalation of chemotherapy/immunosuppressive medications is avoided in the acute phase (e.g., pulse-dose corticosteroids or cyclophosphamide for treating SLE flare). In the majority of patients, symptoms generally resolve in three–eight days.
The MRI can remain abnormal despite improvement in clinical symptoms, although repeat imaging after weeks generally shows resolution of the vasogenic edema. Sometimes, in patients with PRES, there may develop a concurrent intracerebral hemorrhage or ischemic infarction. In these cases, recovery may not be complete.
Our patient had complete neurological recovery within days. She has not had any further seizures in the four months since her presentation, and her anti-epileptic medication has been weaned off. (see Figure 2).
In the interim, a second patient was admitted to our hospital with a similar presentation of septic shock followed by seizures in the setting of relative hypertension, with MRI findings consistent with PRES. She, too, recovered fully with supportive care and BP control. Interestingly, she did not have a history of lupus nephritis, although she did have acute renal failure in the setting of septic shock, and she was only on hydroxychloroquine as an outpatient medication.
We suspect that PRES in patients with SLE may be more common than previously suspected, and we hope that both rheumatologists and neurologists will become more aware of this potential diagnosis in patients with SLE who present with seizures or even just headache. Whether or not it should be considered a form of NPSLE remains a matter of debate, but the importance of BP control (not immunosuppression) is clear.
Anna Helena Jonsson, MD, PhD, is a first-year rheumatology fellow at Brigham and Women’s Hospital in Boston.
Shamik Bhattacharyya, MD, is a senior resident in neurology at Massachusetts General Hospital and Brigham and Women’s Hospital in Boston.
- ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999;42(4):599–608.
- Hanly JG. Diagnosis and management of neuropsychiatric SLE. Nat Rev Rheumatol. 2014;10(6):338–347.
- Karassa FB, Afeltra A, Ambrozic A, et al. Accuracy of anti-ribosomal P protein antibody testing for the diagnosis of neuropsychiatric systemic lupus erythematosus. Arthritis Rheum. 2006;54(1):312–324.
- Fugate JE, Claassen DO, Cloft HJ, et al. Posterior reversible encephalopathy syndrome: Associated clinical and radiologic findings. Mayo Clin Proc. 2010;85(5):427–432.
- Barber CE, Leclerc R, Gladman DD, et al. Posterior reversible encephalopathy syndrome: An emerging disease manifestation in systemic lupus erythematosus. Semin Arthritis Rheum. 2001;41(3):353–363.
- Roth C, Ferbert A. The posterior reversible encephalopathy syndrome: What’s certain, what’s new? Pract Neurol. 2011;11(3):136–144.