Henoch-Schönlein purpura nephritis complicated by reversible posterior leukoencephalopathy syndrome

• Published in: Clinical rheumatology Vol. 26; no. 10; pp. 1761 - 1763
• Main Authors: Sasayama, Daimei, Shimojima, Yasuhiro, Gono, Takahisa, Kaneko, Kazuma, Matsuda, Masayuki, Ikeda, Shu-ichi
• Format: Journal Article
• Language: English
• Published: Germany Springer Nature B.V 01.10.2007
• Subjects: Adolescent; Brain - pathology; Central Nervous System Diseases - complications; Drug therapy; Female; Humans; Hypertension; Hypertension - diagnosis; Hypertension - therapy; Hypertensive Encephalopathy - complications; Hypertensive Encephalopathy - diagnosis; Magnetic Resonance Imaging - methods; Nephritis - complications; Nephritis - diagnosis; Patients; Purpura, Schoenlein-Henoch - complications; Purpura, Schoenlein-Henoch - diagnosis; Seizures; Tomography, X-Ray Computed - methods; Treatment Outcome; Vasculitis
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-006-0502-0

We report a young female patient with Henoch-Schönlein purpura (HSP) nephritis complicated by reversible posterior leukoencephalopathy syndrome (RPLS). The patient suddenly showed generalized seizures and cortical blindness with severe hypertension due to renal insufficiency approximately 1 year after cessation of corticosteroid treatment for HSP nephritis. Magnetic resonance imaging (MRI) demonstrated bilateral abnormal signals mainly in the cerebellum and white matter of the occipital lobe. Clinical symptoms quickly improved in conjunction with disappearance of abnormal signals on brain MRI after starting control of hypertension and continuous hemodiafiltration with steroid pulse therapy and plasmapheresis. RPLS may be caused by vasculitis and also by hemodynamic change due to severe hypertension in HSP, particularly in patients with nephropathy. In such cases intensive treatment should be performed as soon as possible to avoid neurological sequelae.