Abstract: FR-PO547
Different Faces of PRES in Adolescent Lupus Nephritis on Hemodialysis
Session Information
- Dialysis and Vascular Trainee Case Reports
November 08, 2019 | Location: Exhibit Hall, Walter E. Washington Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Report
- 1700 Pediatric Nephrology
Authors
- Mazo, Alexandra, SUNY Downstate Medical Center, Brooklyn, New York, United States
- Mongia, Anil K., SUNY Downstate Medical Center, Brooklyn, New York, United States
- Bamgbola, Oluwatoyin F., SUNY Downstate Medical Center, Brooklyn, New York, United States
Introduction
Posterior reversible encephalopathy syndrome (PRES) is a neurologic condition characterized by seizures, altered mental status, headache, visual changes and specific findings on MRI. For pediatric PRES atypical involvement of frontal lobes, basal ganglia or cerebellum is not rare. Risk factors include SLE, renal disease, dialysis, hypoalbuminemia, hypertension, immunosuppression. Recurrent PRES and status epilepticus (SE) have rarely been described in children.
Case Description
We analysed all cases of PRES in pediatric renal patients in our hospital in the last 5 years and found only 3 cases. All of them are adolescents with active lupus nephritis (LN) on hemodialysis (HD).
Discussion
All our patients had multiple risk factors for PRES: LN, new onset HD, cyclophosphamide, hypoalbuminemia, hypertension. Despite the common predisposing factors, they all had different but atypical course of PRES. Case 1 had PRES one year after an initial lupus cerebritis and had a concurrent pulse therapy following seizure event because of concern for recurring cerebritis. Case 2 had recurrent PRES while she was on nicardipine drip. Case 3 had SE associated with PRES, a very rare condition. Both cases 2 and 3 received the cycle#2 of cyclophosphamide 2 weeks before onset of PRES. However, there was no PRES with the subsequent cycle#3.
Current hypothesis of PRES involves vasogenic edema due to an endothelial injury. Hypertension is a consequence rather than the cause of the disorder; and it may be absent in PRES. LDH was suggested as a useful marker for identification of patients before onset of clinical symptoms. Unfortunately, in our cases LDH was not done. Further studies are needed on the role of LDH as a predictive tool for establishing potential preventive measures such as strict BP control, empirical antiepileptic drug, and optimal fluid control in high risk individuals.
Clinical characteristics during the PRES episode with seizures
| 1st case | 2nd case 1st episode/2nd episode | 3rd case Status Epilepticus | ||
| Age/Sex | 18/F | 16/F | 19/M | |
| LN class | III + V | IV | IV + V | |
| SLE duration | 5 y | 2 y | 3 m | |
| HD duration | 3 m | 1 m | 2 m | |
| Immunosuppression | Azathioprine | Cyclophosphamide #2 | Cyclophosphamide #2 | |
| BP medications | Amlodipine, clonidine, quinapril, minoxidil | Furosemide, nifedipine, labetalol | 1 week after 1st episode on nicardipine drip | Amlodipine, metoprolol, lisinopril |
| BP during PRES | 180/100 | 160/110 | 140/95 | 180/100 |
| PRES area | Frontal, parietal, temporal, occipital lobes | Frontal, parietal, occipital lobes | Thalamus, frontal, parietal, occipital lobes | Frontal, occipital lobes, basal ganglia |
| Antiepileptic medications | levetiracetam* (for 1 y) due to lupus cerebritis, currently on | Levetiracetam started | levetiracetam*, currently on | 2 weeks of valproic acid after PRES, currently without |
| Follow up | 6 m | 2 m | 2 m | |
*questionable adherence