Delayed onset of posterior reversible encephalopathy syndrome in a case of scleroderma renal crisis with maintenance hemodialysis Case report and literature review

作者：Chen Ching Yang; Hung Shin Yuan^*; Lee Yi Jer; Lin Yi Chan; Pai Chu Cheng

来源：Medicine, 2016, 95(52): e5725.

DOI：10.1097/MD.0000000000005725

• case report
• hemodialysis
• microangiopathy
• posterior reversible encephalopathy syndrome
• scleroderma renal crisis
• seizure

Introduction: 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.
Conclusion: 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.

• 出版日期2016-12