36º Congresso Brasileiro de Reumatologia

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Title

POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME (PRES) ASSOCIATED WITH JUVENILE SYSTEMIC ERITEMATOSUS LUPUS: A CASE REPORT

Background

PRES is a neurological disorder characterized by headache, altered mental status, convulsions, ataxia and visual changes. The causes differ between adults and children, being the most common in the pediatric population: renal insufficiency, immunosuppressive therapy, hypertension, and autoimmune diseases such as systemic lupus erythematosus. The pathological mechanism of the disease is based on circulatory changes in the posterior cerebral circulation. There are changes of the image with a predominance of posterior involvement, reversible, similar in all cases, regardless of the primary cause. It does not always reach white matter. Imaging tests such as tomography or magnetic resonance imaging (MRI) show more intense diffuse edema in the parietal and occipital regions bilaterally. The diagnosis has important therapeutic and prognostic implications for the potential reversibility of the lesions if the treatment of the underlying cause is adequately treated.

Case report

JKSN, 12 yeas old, female, active SLE: alopecia, oral ulcers, arthritis, class III nephritis and digital vasculitis, plaquetopenia and low complement. She was hospitalized in January / 2019 for pulsetherapy (4th dose) with methylprednisolone (MP) 1g / day, 3 days and cyclophosphamide 1g. The patient had increased blood pressure (BP), even in the use of captopril, propranolol, spironolactone and furosemide, in optimized doses. During MP infusion she presented paresthesia in the lower left limb, BP of 200x130mmHg, followed by seizure. The MP was discontinued and oxygen therapy support, midazolam IV, furosemide IV, hydralazine was started. There was stabilization of blood pressure. Verified by means of MRI of the brain, areas with high signal in T2 and FLAIR, compromising the cortical / subcortical region of the upper, middle frontal, left pre-central and upper pre-central lobe rotations, associated with small areas of diffusion restriction of the upper parietal lobes, these findings may be related to acute hypertensive encephalopathy (PRES). After controlling for blood pressure levels, pulse therapy was followed without further complications.

Conclusion

PRES is an underdiagnosed pathology and should be considered as a differential diagnosis in neurological manifestations in SLE patients. In the case described, arterial hypertension, MP, LES, lupus nephritis may be considered as triggering factors. The syndrome is a multifactorial entity that needs early detection for an effective approach and neurological recovery.

Área

Pediatrics

Autores

Ludmila Godinho Figueiredo, Victor da Silva Coelho, Diana Campos Fernandino, Paula Reale Fernandes, Vania Schinzel