Posterior Reversible Encephalopathy Syndrome as Presenting Form of Very Early Systemic Sclerosis

Abstract

Introduction. Posterior Reversible Encephalopathy Syndrome (PRES) is an increasingly recognized clinical and radiological entity with a wide spectrum of symptoms. Its mechanism depends on failure of the blood-brain barrier due to high systemic blood pressure (BP) and loss of integrity of vascular endothelium related with different triggers. Methods. We aim to report a case of PRES induced by arterial hypertension and very early systemic sclerosis (SSc) not previously known. Results. A 64-year-old female was admitted due to 1-week pulsating headache more prominent on frontal scalp, accompanied by phonophobia, photophobia, and facial flushing. Neurological exam revealed brisk deep tendon reflex. Brain magnetic resonance imaging (MRI) showed subcortical lesions mainly located in posterior regions. BP was monitored and episodic arterial hypertension was detected. In laboratory tests positive anti-topoisomerase I antibodies were detected. BP was controlled with angiotensin-converting-enzyme inhibitors and headache improved. In a new MRI a month later improvement of white matter lesions was observed. Capillaroscopy showed “active pattern,” considered typical of SSc. Conclusion. In SSc anti-endothelial cell antibodies impair vascular endothelium and liberation of vasoconstrictors leads to BP increasing and disruption of blood-brain barrier autoregulation mechanisms. PRES can be the first manifestation of very early SSc and this entity should be considered even in absence of skin lesions or Raynaud phenomenon.

Etiologies, Cerebral Vasomotion, and Endothelial Dysfunction in the Pathophysiology of Posterior Reversible Encephalopathy Syndrome in Pediatric Patients

“…An increased prevalence of PRES occurring in patients with connective tissue disorders and vasculitides, including systemic lupus erythematosus (SLE), scleroderma, 269,270 Sjogren's syndrome, 143 juvenile idiopathic arthritis, 144 macrophage activation syndrome, 145 Takayasu's arteritis, 147 polyarteritis nodosa, 148 Wegener's granulomatosis, 149 and Henoch-Schönlein purpura, 150 could alternatively implicate renal dysfunction and azotemia, steroid or immunosuppressive therapy, or dysregulation of immune mechanisms in the pathogenesis of this condition. 73,271,272 Accordingly, Raj and colleagues 69,70 presented a cohort of children developing PRES, demonstrating an approximately 20% prevalence of SLE among these patients.…”