title: Delayed onset of posterior reversible encephalopathy syndrome in a case of scleroderma renal crisis with maintenance hemodialysis: Case report and literature review.
abstract: BACKGROUND
  In some cases, scleroderma renal crisis (SRC) is not easily distinguishable from other thrombotic microangiopathies such as thrombotic thrombocytopenic purpura, especially when the presentation includes neurological or extra-renal manifestations. Here, we present a case of SRC who developed a rare neurotoxic complication, posterior reversible encephalopathy syndrome (PRES).A 36-year-old man with a history of diffuse cutaneous systemic sclerosis developed SRC and acute-on-chronic renal failure and ultimately required maintenance hemodialysis. Three weeks after starting hemodialysis, the patient presented with confusion and a new-onset seizure disorder. Laboratory examinations revealed thrombocytopenia, a low haptoglobin level, and schizocytes on a blood smear. SRC-related PRES was considered first after PRES was confirmed by brain magnetic resonance imaging. Antihypertensive therapy comprising captopril and amlodipine was administered, and the patient experienced a complete neurological recovery 3 days later without plasma exchange. In all previously reported cases of SRC-associated PRES, PRES developed before hemodialysis. Our report is, therefore, the first to describe a case of onset of SRC-related PRES 3 weeks after the initiation of maintenance hemodialysis.
  
  
  CONCLUSIONS
  This case demonstrates that microangiopathy and extra-renal manifestations can develop even in SRC patients with end-stage renal disease and that these manifestations can be successfully managed with angiotensin-converting enzyme inhibitors (ACEIs) and aggressive blood pressure control. We recommend continuing ACEI therapy if elevated blood pressure persists after maintenance hemodialysis.
authors.affiliation: Division of Nephrology, Department of Internal Medicine, E-DA Hospital, No. 1, Yida Road, Jiaosu Village, Yanchao District, Kaohsiung City 82445, Taiwan, R.O.C School of Medicine for International Students, I-Shou University, No. 8, Yida Rd., Jiaosu Village Yanchao District, Kaohsiung City 82445, Taiwan, R.O.C Division of Nephrology, Department of Internal Medicine, E-DA Cancer Hospital, No. 21, Yida Road, Jiaosu Village, Yanchao District, Kaohsiung City 82445, Taiwan, R.O.C.
authors.forename: Ching-Yang
authors.initials: CY
authors.lastname: Chen
authors.forename: Shin-Yuan
authors.initials: SY
authors.lastname: Hung
authors.forename: Yi-Jer
authors.initials: YJ
authors.lastname: Lee
authors.forename: Yi-Chan
authors.initials: YC
authors.lastname: Lin
authors.forename: Chu-Cheng
authors.initials: CC
authors.lastname: Pai
mesh_terms: D058186:Acute Kidney Injury / Q000150:complications / Q000209:etiology*; D000328:Adult; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D059906:Neuroimaging; D054038:Posterior Leukoencephalopathy Syndrome / Q000000981:diagnostic imaging / Q000209:etiology*; D006435:Renal Dialysis; D012595:Scleroderma, Systemic / Q000150:complications*