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The challenging clinical dilemma of posterior reversible encephalopathy syndrome in systemic lupus erythematosus

Das herausfordernde klinische Dilemma des Syndroms der posterioren reversiblen Enzephalopathie bei systemischem Lupus erythematosus

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Abstract

Background and objective

Posterior reversible encephalopathy syndrome (PRES) in systemic lupus erythematosus (SLE) is a challenging clinical dilemma. A retrospective single-center study was performed to investigate the clinical features, risk factors, outcomes, and clinical determinants of the prognosis of PRES in SLE.

Methods

A retrospective study was performed from January 2015 to December 2020. 19 episodes of lupus PRES and 19 episodes of non-lupus PRES were identified. 38 cases of patients presenting with neuropsychiatric lupus (NPSLE) hospitalized during the same period were selected as controls. Survival status was acquired via outpatient and telephone follow-up in December 2022.

Results

The clinical neurological presentation of PRES in lupus patients was similar to that of the non-SLE-related PRES and NPSLE populations. Nephritis-induced hypertension is the predominant trigger of PRES in SLE. Disease flare and renal failure-triggered PRES were identified in half of the patients with SLE. The mortality rate of lupus-related PRES during the 2‑year follow-up was 15.8%, the same as that of NPSLE. For patients with lupus-related PRES, multivariate analysis indicated that high diastolic blood pressure (OR =1.762, 95% CI: 1.031 ~ 3.012, p = 0.038), renal involvement (OR = 3.456, 95% CI: 0.894 ~ 14.012, p = 0.049), and positive proteinuria (OR = 1.231, 95% CI: 1.003 ~ 1.511, p = 0.047) were independent risk factors compared to NPSLE. A strong connection between the absolute counts of T and/or B cells and prognosis in lupus patients with neurological manifestations was found (p < 0.05). The lower the counts of T and/or B cells, the worse the prognosis.

Conclusion

Lupus patients with renal involvement and disease activity are more likely to develop PRES. The mortality rate of lupus-related PRES is similar to that of NPSLE. Focusing on immune balance might reduce mortality.

Zusammenfassung

Hintergrund und Ziel

Das Syndrom der posterioren reversiblen Enzephalopathie (PRES) bei systemischem Lupus erythematosus (SLE) stellt ein klinisches Dilemma und somit eine Herausforderung dar. In einer retrospektiven Einzelzentrumstudie wurden nun die klinischen Merkmale, Risikofaktoren, Ergebnisse und klinische Parameter für die Prognose des PRES bei SLE untersucht.

Methoden

Dazu wurde von Januar 2015 bis Dezember 2020 eine retrospektive Studie durchgeführt. Es wurden 19 Episoden von Lupus-PRES und 19 Episoden von Non-Lupus-PRES identifiziert. Als Kontrollen wurden 38 Fälle von Patienten ausgewählt, die sich mit neuropsychiatrischem Lupus (NPSLE) vorstellten und während des gleichen Zeitraums stationär aufgenommen wurden. Im Dezember 2022 wurde bei ambulanten und telefonischen Nachsorgeterminen der Überlebensstatus erhoben.

Ergebnisse

Die klinisch-neurologischen Manifestationen des PRES bei Lupuspatienten waren ähnlich wie bei den Gruppen mit Non-Lupus-PRES und NPSLE. Vorherrschender Trigger des Lupus-PRES ist die nephritisinduzierte Hypertonie. Ein Schub der Erkrankung und ein durch Niereninsuffizienz getriggertes PRES wurden bei der Hälfte der Patienten mit SLE festgestellt. Die Mortalitätsrate des Lupus-PRES während des 2‑Jahres-Follow-up betrug 15,8%, der gleiche Wert wie bei NPSLE. Für Patienten mit Lupus-PRES zeigte die multivariate Analyse, dass ein hoher diastolischer Blutdruck (Odds Ratio, OR=1,762; 95%-Konfidenzintervall, 95%-KI: 1,031 ~ 3,012; p = 0,038), Nierenbeteiligung (OR = 3,456; 95%-KI: 0,894 ~ 14,012; p = 0,049) und Vorliegen einer Proteinurie (OR = 1,231; 95%-KI: 1,003 ~ 1,511; p = 0,047) unabhängige Risikofaktoren im Vergleich zum NPSLE darstellten. Es wurde ein starker Zusammenhang zwischen den absoluten Werten für T‑ und/oder B‑Zellen und der Prognose bei Lupuspatienten mit neurologischen Manifestationen festgestellt (p < 0,05). Je niedriger die Werte für T‑ und/oder B‑Zellen, umso schlechter die Prognose.

Schlussfolgerung

Lupuspatienten mit Nierenbeteiligung und Krankheitsaktivität weisen eine höhere Wahrscheinlichkeit für die Entstehung eines PRES auf. Die Mortalitätsrate des Lupus-PRES ist ähnlich wie die des NPSLE. Eine Fokussierung auf das Immungleichgewicht könnte die Mortalität reduzieren.

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Acknowledgements

The first author thanks her husband, Qiyuan Cui, for valuable help and contribution.

Funding

This work was supported by grants from the National Natural Science Foundation (81871292) and the Key Research and Development (R&D) Projects of Shanxi Province (201803D31136).

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Authors and Affiliations

  1. Department of Rheumatology and Immunology, Shanxi Bethune Hospital, Shanxi Academy of Medical Sciences, Tongji Shanxi Hospital, Third Hospital of Shanxi Medical University, 030032, Taiyuan, China

    Yang Liu, Qian Li, Ying Liu, Pengyan Qiao, Sumiao Liu & Ke Xu

  2. Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, 430030, Wuhan, China

    Yang Liu, Qian Li, Ying Liu, Pengyan Qiao, Sumiao Liu & Ke Xu

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  1. Yang Liu
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  2. Qian Li
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Corresponding author

Correspondence to Ke Xu.

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Conflict of interest

Y. Liu, Q. Li, Y. Liu, P. Qiao, S. Liu, and K. Xu have no conflicts of interest to declare.

For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case.

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Uwe Lange, Bad Nauheim

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Liu, Y., Li, Q., Liu, Y. et al. The challenging clinical dilemma of posterior reversible encephalopathy syndrome in systemic lupus erythematosus. Z Rheumatol 83 (Suppl 1), 124–131 (2024). https://doi.org/10.1007/s00393-023-01385-8

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