Cargando…

A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare

Posterior reversible encephalopathy syndrome (PRES) is a syndrome encompassing both clinical and radiological manifestations with white matter vasogenic edema predominantly of the posterior and parietal lobes of the brain. It may accompany several medical conditions including immunosuppressive/cytot...

Descripción completa

Detalles Bibliográficos
Autores principales: Tabot Tabot, Mpey K, Ababio, Priscilla A, Waldron, Shervonne, Rougui, Lamiaa, Mehari, Alem
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9976264/
https://www.ncbi.nlm.nih.gov/pubmed/36874730
http://dx.doi.org/10.7759/cureus.34372
_version_ 1784899023106211840
author Tabot Tabot, Mpey K
Ababio, Priscilla A
Waldron, Shervonne
Rougui, Lamiaa
Mehari, Alem
author_facet Tabot Tabot, Mpey K
Ababio, Priscilla A
Waldron, Shervonne
Rougui, Lamiaa
Mehari, Alem
author_sort Tabot Tabot, Mpey K
collection PubMed
description Posterior reversible encephalopathy syndrome (PRES) is a syndrome encompassing both clinical and radiological manifestations with white matter vasogenic edema predominantly of the posterior and parietal lobes of the brain. It may accompany several medical conditions including immunosuppressive/cytotoxic drugs. We present a case of cyclophosphamide-induced PRES in a patient treated for acute lupus flare with biopsy-proven lupus nephritis. A 23-year-old African American female presented with non-specific symptoms over a six-month period on a medical background of systemic lupus erythematosus and biopsy-proven focal lupus nephritis class III on hydroxychloroquine, prednisone, and mycophenolate mofetil for which she was non-compliant. She was borderline hypertensive, tachycardic, saturating well on ambient air, and alert and oriented. Laboratory workup revealed electrolyte imbalance, elevated serum urea, creatinine, and B-type natriuretic peptide, low serum complements, and elevated double-stranded DNA (dsDNA) with negative lupus anticoagulant, anti-cardiolipin, and B2 glycoprotein antibody. Chest imaging revealed cardiomegaly with small pericardial effusion, left pleural effusion, and trace atelectasis, with no deep vein thrombosis on Doppler ultrasound. She was admitted to the intensive care unit for lupus flare with severe hyponatremia and was continued on mycophenolate mofetil, hydroxychloroquine, and prednisone 60 mg for induction therapy as well as intravenous fluids. Hyponatremia resolved, and blood pressure was controlled. She became fluid overloaded and anuric, with pulmonary edema and worsening hypoxic respiratory failure not responding to diuretic challenges. Daily hemodialysis was started, and she was intubated. Prednisone was tapered down, mycophenolate was switched to cyclophosphamide/mesna. She became agitated, restless, and confused, with waxing and waning consciousness and hallucinations. She was continued on bi-weekly cyclophosphamide for induction therapy. After the second dose of cyclophosphamide, her mentation worsened. Non-contrast MRI showed extensive bilateral cerebral and cerebella deep white matter high-intensity signals suggestive of PRES, which was new compared to one year prior. Cyclophosphamide was held and her mentation improved. She was successfully extubated and discharged to a rehabilitation center. The exact pathophysiological mechanism of PRES is not known. Endothelial damage and vasogenic edema have been hypothesized as possible mechanisms. Severe anemia, fluid overload, and renal failure are some of the causes of endothelial dysfunction and vasogenic edema with disruption of the blood-brain barrier, which were found in our patient, but repeated dosing of cyclophosphamide worsened her condition. Discontinuation of cyclophosphamide led to a significant improvement and complete reversal of her neurologic symptoms, implying that prompt recognition and management of PRES is vital to prevent permanent damage and even death in these patients.
format Online
Article
Text
id pubmed-9976264
institution National Center for Biotechnology Information
language English
publishDate 2023
publisher Cureus
record_format MEDLINE/PubMed
spelling pubmed-99762642023-03-02 A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare Tabot Tabot, Mpey K Ababio, Priscilla A Waldron, Shervonne Rougui, Lamiaa Mehari, Alem Cureus Internal Medicine Posterior reversible encephalopathy syndrome (PRES) is a syndrome encompassing both clinical and radiological manifestations with white matter vasogenic edema predominantly of the posterior and parietal lobes of the brain. It may accompany several medical conditions including immunosuppressive/cytotoxic drugs. We present a case of cyclophosphamide-induced PRES in a patient treated for acute lupus flare with biopsy-proven lupus nephritis. A 23-year-old African American female presented with non-specific symptoms over a six-month period on a medical background of systemic lupus erythematosus and biopsy-proven focal lupus nephritis class III on hydroxychloroquine, prednisone, and mycophenolate mofetil for which she was non-compliant. She was borderline hypertensive, tachycardic, saturating well on ambient air, and alert and oriented. Laboratory workup revealed electrolyte imbalance, elevated serum urea, creatinine, and B-type natriuretic peptide, low serum complements, and elevated double-stranded DNA (dsDNA) with negative lupus anticoagulant, anti-cardiolipin, and B2 glycoprotein antibody. Chest imaging revealed cardiomegaly with small pericardial effusion, left pleural effusion, and trace atelectasis, with no deep vein thrombosis on Doppler ultrasound. She was admitted to the intensive care unit for lupus flare with severe hyponatremia and was continued on mycophenolate mofetil, hydroxychloroquine, and prednisone 60 mg for induction therapy as well as intravenous fluids. Hyponatremia resolved, and blood pressure was controlled. She became fluid overloaded and anuric, with pulmonary edema and worsening hypoxic respiratory failure not responding to diuretic challenges. Daily hemodialysis was started, and she was intubated. Prednisone was tapered down, mycophenolate was switched to cyclophosphamide/mesna. She became agitated, restless, and confused, with waxing and waning consciousness and hallucinations. She was continued on bi-weekly cyclophosphamide for induction therapy. After the second dose of cyclophosphamide, her mentation worsened. Non-contrast MRI showed extensive bilateral cerebral and cerebella deep white matter high-intensity signals suggestive of PRES, which was new compared to one year prior. Cyclophosphamide was held and her mentation improved. She was successfully extubated and discharged to a rehabilitation center. The exact pathophysiological mechanism of PRES is not known. Endothelial damage and vasogenic edema have been hypothesized as possible mechanisms. Severe anemia, fluid overload, and renal failure are some of the causes of endothelial dysfunction and vasogenic edema with disruption of the blood-brain barrier, which were found in our patient, but repeated dosing of cyclophosphamide worsened her condition. Discontinuation of cyclophosphamide led to a significant improvement and complete reversal of her neurologic symptoms, implying that prompt recognition and management of PRES is vital to prevent permanent damage and even death in these patients. Cureus 2023-01-30 /pmc/articles/PMC9976264/ /pubmed/36874730 http://dx.doi.org/10.7759/cureus.34372 Text en Copyright © 2023, Tabot Tabot et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Tabot Tabot, Mpey K
Ababio, Priscilla A
Waldron, Shervonne
Rougui, Lamiaa
Mehari, Alem
A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title_full A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title_fullStr A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title_full_unstemmed A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title_short A Rare Case of Cyclophosphamide-Induced Posterior Reversible Encephalopathy Syndrome in a Patient With Acute Lupus Nephritis Flare
title_sort rare case of cyclophosphamide-induced posterior reversible encephalopathy syndrome in a patient with acute lupus nephritis flare
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9976264/
https://www.ncbi.nlm.nih.gov/pubmed/36874730
http://dx.doi.org/10.7759/cureus.34372
work_keys_str_mv AT tabottabotmpeyk ararecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT ababiopriscillaa ararecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT waldronshervonne ararecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT rouguilamiaa ararecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT meharialem ararecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT tabottabotmpeyk rarecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT ababiopriscillaa rarecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT waldronshervonne rarecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT rouguilamiaa rarecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare
AT meharialem rarecaseofcyclophosphamideinducedposteriorreversibleencephalopathysyndromeinapatientwithacutelupusnephritisflare