A Rare Neurological Complication - Posterior Reversible Encephalopathy Syndrome in a Patient with Systemic Lupus Erythematosus and Class IV Lupus Nephritis

Open access

Abstract

Posterior reversible encephalopathy syndrome is a rare manifestation of systemic lupus erythematosus, characterized by altered mental status, headache, convulsions, visual field impairment and posterior and reversible alterations on imaging scans(1,2). The clinical picture develops over a few hours, presenting with rapidly progressive neurological symptoms(3). It was first described in 1996. It is more frequent in patients with acute kidney injury or chronic kidney disease, thus in lupus patients with kidney disorders. It is associated with hypertension, other autoimmune diseases beside lupus, immunosuppressive therapies, especially antibody-based immunosuppressive therapy, and organ transplantation. It is clinically reversible within one week and imaging changes resolve within 2-4 weeks. It is treatable and has a good prognosis. We present the case of a young woman of 27 years, diagnosed with systemic lupus erythematosus who developed convulsive seizures, headache, visual impairment, being under immunosuppressive therapy with azathioprine. The kidney biopsy revealed class IV lupus nephritis and partial remission of the nephrotic syndrome. The other manifestations of SLE in this patient were cutaneous, immunological, articular and haematological. The patient had a good short, medium and long-term prognosis at 30 days and also at 6 months.

1. Hinchey J, Chaves C, Appignani B, Breen J, Pao L, et al. (1996) A reversible posterior leukoencephalopathy syndrome. N Engl J Med 334(8): 494-500.

2. McKinney AM, Short J, Truwit CL, McKinney ZJ, Kozak OS, et al. (2007) Posterior reversible encephalopathy syndrome: incidence of atypical regions of involvement and imaging findings. AJR Am J Roentgenol 189(4): 904-912.

3. Gatla N, Annapureddy N, Sequeira W, Jolly M (2013) Posterior Reversible Encephalopathy Syndrome in Systemic Lupus Erythematosus. J Clin Rheumatol 19(6): 334-340.

4. Kur JK, Esdaile JM (2006) Posterior reversible encephalopathy syndrome-an underrecognized manifestation of systemic lupus erythematosus. J Rheumatol 33(11): 2178-2183.

5. Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 1: fundamental imaging and clinical features. AJNR Am J Neuroradiol 29(6): 1036-1042.

6. Bartynski WS (2008) Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol 29(6): 1043-1049.

7. Chien-Chih Lai, Wei-Sheng Chen, Yu-Sheng Chang, Shu-Hung Wang, Chun-Jui et al. (2013) Clinical Features and Outcomes of Posterior Reversible Encephalopathy Syndrome in Patients With Systemic Lupus Erythematosus. Arthritis Care & Research (Roboken) 65(11): 17661774.

8. Fugate JE, Rabinstein AA (2015) Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions. Lancet Neurol 14(9): 914-925.

9. Casey SO, Sampaio RC, Michel E, Truwit CL (2000) Posterior reversible encephalopathy syndrome: utility of fluid-attenuated inversion recovery MR imaging in the detection of cortical and subcortical lesions. AJNR Am J Neuroradiol 21(7): 1199-1206.

10. Burnett MM, Hess CP, Roberts JP, Bass NM, Douglas VC, (2010) Presentation of reversible posterior leukoencephalopathy syndrome in patients on calcineurin inhibitors. Clin Neurol Neurosurg 112(10): 886-889.

11. Kozak OS, Wijdicks EF, Manno EM, Miley JT, Rabinstein AA (2007) Status epilepticus as initial manifestation of posterior reversible encephalopathy syndrome. Neurology 69(9): 894-897.

12. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA (2008) Clinical spectrum of reversible posterior leukoencephalopathy syndrome. Arch Neurol 65(2): 205-210.

13. Legriel S, Pico f, Azoulay E (2011) Understanding Posterior Reversible Encephalopathy Syndrome. Vincent JL (Ed.), Annual Update in Intensive Care and Emergency Medicine 2011: 631-653.

14. Shaharir SS, Remli R, Marwan AA, Said MS, Kong NC (2013) Posterior reversible encephalopathy syndrome in systemic lupus erythematosus: pooled analysis of the literature reviews and report of six new cases. Lupus 22(5): 492-496.

15. Schattner A, Al-Bewerat A. Levetiracetam (Keppra) (2016) Urinary retention and literature search. The Netherlands Journal of Medicine 74(8): 371.

16. Leroux G, Sellam J, Costedoat-Chalumeau N, Le Thi Huong D, et al. (2008) Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature. Lupus 17(2):139-147.

17. Gatla N, Annapureddy N, Sequeira W, Jolly M (2013) Posterior reversible encephalopathy syndrome in systemic lupus erythematosus. J Clin Rheumatol 19(6): 334-340.

18. Lee VH, Wijdicks EF, Manno EM, Rabinstein AA. Clinical spectrum of reversible posterior leukoencephalopathy syndrome.Arch Neurol. 2008;65:205–210

19. Schattner A, Al-Bewera A, Levetiracetam (Keppra), urinary retention and literature search. The Ned J Med. 2016, 74: 371

Journal Information

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 81 81 10
PDF Downloads 44 44 7