Reversible posterior leukoencephalopathy syndrome in a patient presenting granulomatosis with polyangiitis

• Published in: Annales de dermatologie et de vénéréologie Vol. 144; no. 2; pp. 113 - 120
• Main Authors: Chaput, L, Rabot, N, Limousin, N, Cottier, J-P, Lioger, B, Samimi, M
• Format: Journal Article
• Language: French
• Published: France 01.02.2017
• Subjects: Adrenal Cortex Hormones - therapeutic use; Anticonvulsants - therapeutic use; Antihypertensive Agents - therapeutic use; Brain - drug effects; Brain - pathology; Cyclophosphamide - therapeutic use; Female; Granulomatosis with Polyangiitis - diagnosis; Granulomatosis with Polyangiitis - drug therapy; Humans; Magnetic Resonance Imaging; Methylprednisolone - adverse effects; Methylprednisolone - therapeutic use; Neurologic Examination - drug effects; Posterior Leukoencephalopathy Syndrome - chemically induced; Posterior Leukoencephalopathy Syndrome - diagnosis; Posterior Leukoencephalopathy Syndrome - drug therapy; Risk Factors; White Matter - drug effects; White Matter - pathology; Young Adult; Granulomatosis with polyangiitis; Posterior reversible encephalopathy syndrome; Systemic vasculitis; Vascularite; Syndrome d’encéphalopathie postérieure reversible; Granulomatose avec polyangéite
• ISSN: 0151-9638
• DOI: 10.1016/j.annder.2016.06.012

Reversible posterior leukoencephalopathy syndrome (RPLS) is characterised by clinical neurological features of sudden onset and brain MRI findings such as T2/Flair white matter hyperintensities. RPLS can occur in autoimmune diseases, and rarely in systemic vasculitis. We report a case of RPLS in a woman presenting granulomatosis with polyangiitis (Wegener's granulomatosis). A 22-year-old female patient was treated with methylprednisolone pulses for granulomatosis with polyangiitis and neurological impairment. A few hours after the second pulse, the patient had seizures, blindness and confusion associated with high blood pressure and acute renal failure. MRI revealed a high-intensity area on T2-Flair weighted images of the occipital-temporal lobes. The patient was treated with antiepileptic and antihypertensive medications, oral steroids and cyclophosphamide; the clinical and radiological findings proved reversible over the ensuing days. The occurrence of RPLS in systemic vasculitis is rare. Six cases of RPLS associated with granulomatosis and polyangiitis have been reported. It appears important to screen for high blood pressure in patients recently treated with corticosteroids for vasculitis as this condition may represent a precipitating factor for RPLS.