Clinical profile and risk factors of posterior reversible encephalopathy syndrome in systemic lupus erythematosus: A case-control study

Abstract

Objectives

To investigate the clinical profile and risk factors of Posterior Reversible Encephalopathy Syndrome (PRES) in patients with Systemic Lupus Erythematosus (SLE).

Methods

In this retrospective study, the SLE patients admitted with PRES in the Department of Clinical Immunology and Rheumatology, IPGME&R, Kolkata, India from January 1, 2017 to January 31, 2024, were identified. Fifty-three control participants with neuropsychiatric symptoms as per the 1999 American College of Rheumatology case definition criteria were selected from the departmental SLE cohort.

Results

26 episodes of PRES were identified in 25 patients. Seizures (88.46%) and headaches (53.85%) were the commonest presenting manifestations with the occipital (96.15%) and parietal lobes (84.62%) being the commonly involved sites. Nephritis (96.15% vs 60.38%, p = 0.001), hypertension at PRES onset (76.92% vs 18.87%, p = 0.000), Direct Coomb’s test (DCT) positivity (50.00% vs 20.75%, p = 0.008), high extra-neurological disease activity (SELENA-SLEDAI – N: 20.62±8.77 vs 14.15 ± 5.51, p = 0.002), lupus enteritis (15.38% vs 1.89%, p = 0.038), and neutrophilia (73.08% vs 20.75%, p = 0.000) were identified as risk factors for PRES on univariate analysis. Multivariate analysis found hypertension [p = 0.014], DCT positivity (p = 0.013), neutrophilia (p = 0.025), and lupus enteritis (0.038) as independent risk factors. The presence of U1RNP/Sm and prior thrombotic events were found to be protective against PRES on univariate analysis.

Conclusion

PRES in SLE in our study was associated with high extra-neurological disease activity. Hypertension, DCT positivity, neutrophilia, and lupus enteritis were identified as important risk factors for the condition.

Keywords

Posterior reversible encephalopathy syndrome PRES neuropsychiatric lupus subacute lupus erythematosus

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References

Hinchey

Chaves

Appignani

, et al. A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996; 334: 494–500.

Fugate

Claassen

Cloft

, et al. Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings. Mayo Clin Proc 2010; 85: 427–432.

Marik

. Hypertensive disorders of pregnancy. Postgrad Med J 2009; 121: 69–76.

Schwartz

Mulkern

Gudbjartsson

, et al. Diffusion-weighted MR imaging in hypertensive encephalopathy: clues to pathogenesis. AJNR Am J Neuroradiol 1998; 19: 859–862.

Punaro

Abou-Jaoude

Cimaz

, et al. Unusual neurologic manifestations (II): posterior reversible encephalopathy syndrome (PRES) in the context of juvenile systemic lupus erythematosus. Lupus 2007; 16: 576–579.

Bartynski

Boardman

Zeigler

, et al. Posterior reversible encephalopathy syndrome in infection, sepsis, and shock. AJNR Am J Neuroradiol 2006; 27: 2179–2190.

Nakabou

Kai

Maeshima

, et al. Hypertensive encephalopathy in patients with chronic renal failure caused by stopping antihypertensive agents: a report of two cases. Clin Exp Nephrol 2010; 14: 256–262.

Wijdicks

. Neurotoxicity of immunosuppressive drugs. Liver Transpl 2001; 7: 937–942.

Huang

Tsai

Yeh

, et al. Reversible posterior leukoencephalopathy syndrome caused by blood transfusion: a case report. Acta Neurol Taiwan 2008; 17: 258–262.

10.

Bartynski

. Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol 2008; 29: 1036–1042.

11.

Lai

Chen

Chang

, et al. Clinical features and outcomes of posterior reversible encephalopathy syndrome in patients with systemic lupus erythematosus. Arthritis Care Res 2013; 65: 1766–1774.

12.

The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999; 42: 599–608.

13.

Liu

Zhang

, et al. Posterior reversible encephalopathy syndrome could be an underestimated variant of ‘‘reversible neurological deficits’’ in systemic lupus erythematosus. BMC Neurol 2012; 12: 152.

14.

Cui

Lei

Zhang

, et al. Clinical features, outcomes and risk factors for posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a case-control study. Lupus 2019; 28(8): 961–969.

15.

Damrongpipatkul

Oranratanachai

Kasitanon

, et al. Clinical features, outcome, and associated factors for posterior reversible encephalopathy in Thai patients with systemic lupus erythematosus: a case-control study. Clin Rheumatol 2018; 37: 691–702.

16.

Baizabal-Carvallo

Barragán-Campos

Padilla-Aranda

, et al. Posterior reversible encephalopathy syndrome as a complication of acute lupus activity. Clin Neurol Neurosurg 2009; 111: 359–363.

17.

Merayo-Chalico

Apodaca

Barrera-Vargas

, et al. Clinical outcomes and risk factors for posterior reversible encephalopathy syndrome in systemic lupus erythematosus: a multicentric case-control study. J Neurol Neurosurg Psychiatry 2016; 87(3): 287–294.

18.

Hochberg

. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 1997; 40(9): 1725.

19.

Fischer

Schmutzhard

. Posterior reversible encephalopathy syndrome. J Neurol 2017; 264(8): 1608–1616.

20.

Bortoluzzi

Scirè

Bombardieri

, et al. Development and validation of a new algorithm for attribution of neuropsychiatric events in systemic lupus erythematosus. Rheumatology 2015; 54(5): 891–898.

21.

Petri

Kim

Kalunian

OC-SELENA and Trial , et al.. Combined oral contraceptives in women with systemic lupus erythematosus. N Engl J Med 2005; 353(24): 2550–2558.

22.

Varaprasad

Agrawal

Prabu

, et al. Posterior reversible encephalopathy syndrome in systemic lupus erythematosus. J Rheumatol 2011; 38(8): 1607–1611.

23.

Jung

Moon

Kwok

, et al. Posterior reversible encephalopathy syndrome in Korean patients with systemic lupus erythematosus: risk factors and clinical outcome. Lupus 2013; 22: 885–891.

24.

Shaharir

Remli

Marwan

, et al. Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: pooled analysis of the literature reviews and report of six new cases. Lupus 2013; 22(5): 492–496.

25.

Budhoo

Mody

. The spectrum of posterior reversible encephalopathy in systemic lupus erythematosus. Clin Rheumatol 2015; 34(12): 2127–2134.

26.

Fugate

Rabinstein

. Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 2015; 14: 914–925.

27.

Marra

Vargas

Striano

, et al. Posterior reversible encephalopathy syndrome: the endothelial hypotheses. Med Hypotheses. 2014; 82: 619–622.

28.

Pai

Hegde

Nair

, et al. Adult primary intraventricular hemorrhage: clinical characteristics and outcomes. J Neurosci Rural Pract 2020; 11(4): 623–628.

29.

Buttar

Lakhdar

Castillo

, et al. Cyclophosphamide induced posterior reversible encephalopathy syndrome (PRES) in a patient with lupus nephritis. Clin Med Rev Case Rep 2021; 8: 354.

30.

Jabrane

Ait Lahcen

Fadili

, et al. A case of PRES in an active lupus nephritis patient after treatment of corticosteroid and cyclophosphamide. Rheumatol Int 2015; 35(5): 935–938.

31.

Zekić

Benić

Antulov

, et al. The multifactorial origin of posterior reversible encephalopathy syndrome in cyclophosphamide-treated lupus patients. Rheumatol Int 2017; 37(12): 2105–2114.

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