Posterior reversible encephalopathy syndrome as a complication of Henoch–Schönlein purpura in a seven-year-old girl

Abstract

Introduction Henoch-Schönlein purpura is a multisystem small vessel vasculitis. Neurologic manifestations are uncommon. Posterior reversible encephalopathy syndrome is a rare complication of Henoch-Schönlein purpura with typical clinical and neuroimaging findings that occurs most commonly in the setting of severe hypertension and renal injury. Case presentation A seven-year-old girl was admitted to our institution presenting with clinical and laboratory findings suggestive of Henoch-Schönlein purpura. Glucocorticoid therapy was initiated, but five days following her admission, she developed altered consciousness, seizures, arterial hypertension, and cortical blindness. Brain MRI scan revealed areas of vasogenic oedema in parieto-occipital lobes, consistent with posterior reversible encephalopathy syndrome. She was immediately initiated on antihypertensives and antiepileptics, which successfully improved her neurologic symptoms. Further laboratory work-up disclosed a rapidly progressive glomerulonephritis secondary to Henoch-Schönlein purpura that was the likely cause of her sudden blood pressure elevation. Immunosuppressive therapy was undertaken, and at one-year follow-up, the patient exhibited complete renal and neurologic recovery. Conclusion Posterior reversible encephalopathy syndrome is a severe complication of Henoch-Schönlein purpura. If promptly diagnosed and treated, children with Henoch-Schönlein purpura presenting with posterior reversible encephalopathy syndrome usually have a good prognosis. Clinicians should be familiar with the characteristic presentation of posterior reversible encephalopathy syndrome and be aware that hypertension and renal injury may predispose Henoch-Schönlein purpura patients to developing this complication.

Posterior reversible encephalopathy syndrome with Lilliputian hallucinations secondary to Takayasu's arteritis

“…PRES is often associated with hypertension, renal disease, rheumatological conditions, and some immunosuppressive medications [4 , 6 , 7] . Typical findings on MRI brain imaging show vasogenic oedema in the affected lobes, [5 , 7] most frequently the occipital lobes, followed by the parietal, frontal, and temporal lobes [7] .…”

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

“…7 Chemotherapeutic agents may accordingly contribute to the pathogenesis of PRES by directly effecting osmotic injury, necrosis or apoptosis of endothelial cells, 14,47,50,52,58,105,115,122,125 or inducing axonal swelling. 14,58 Sans direct cytotoxic injury, a variety of etiologies may converge on augmenting infectious 11,[126][127][128][129][130][131][132][133][134][135][136][137][138][139][140] or autoimmune [141][142][143][144][145][146][147][148][149][150] mediated activation of cellular adaptive immune mechanisms and pathways, effecting the activation of macrophages and consequent elaboration of cytokines, including tumor necrosis factor α, interleukin-1, interleukin-6, interleukin-8, and interleukin-12. 151,152 Collectively, these molecules generate fever by promoting the synthesis of pyrogenic prostaglandins in the thermoregulatory nuclei of the hypothalamus and upregulate the expression of vascular cell adhesion molecule 1 (VCAM-1) and intracellular adhesion molecule 1 (ICAM-1) in the endothelium.…”

“…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.…”

IgA vasculitis, formerly known as Henoch-Schönlein purpura – the most common vasculitis in children

“…In the course of the disease, we can also observe symptoms of the nervous system (< 1% of cases in children): headaches, behavioural disorders, visual and speech disorders, disturbances in consciousness, convulsions, and focal symptoms. Several cases of Posterior Reversible Encephalopathy Syndrome (PRES) have been described, which may be the result of inflammation of the nervous system vessels or hypertension [53]. Hypertension that is observed in patients with HSP may lead to central nervous system (CNS) haemorrhage.…”