Posterior reversible encephalopathy syndrome and microangiopathic haemolytic anaemia developing in a regularly haemodialysed patient with scleroderma renal crisis: a case report

Abstract

Scleroderma renal crisis (SRC) is a severe complication of systemic sclerosis characterised by abrupt onset of arterial hypertension and rapid progression to renal failure. Angiotensin-converting enzyme inhibitor (ACEi) is widely used for the treatment of this disease. Although the prognosis of SRC can be improved by prompt initiation of ACEi, a considerable proportion of cases still require maintenance dialysis therapy. However, the clinical picture of SRC patients after introduction of haemodialysis has not been sufficiently investigated. Here, we describe a 69-year-old female patient who was receiving regular haemodialysis because of SRC for three months and who developed posterior reversible encephalopathy syndrome (PRES) or microangiopathic haemolytic anaemia (MAHA), accompanied by rapid elevation of blood pressure, shortly after discontinuation of ACEi. Resumption of ACEi quickly resulted in improvement of the patient’s condition and allowed stable continuation of maintenance haemodialysis. As haemodialysis patients often develop hypotension during dialysis sessions, reduction of antihypertensive agents need to be considered. However, physicians must decide carefully whether antihypertensive drugs including ACEi should be discontinued in order to avoid complications such as PRES or MAHA related to blood pressure elevation during the management of patients receiving maintenance haemodialysis for SRC.

Keywords:

• Scleroderma renal crisis
• haemodialysis
• posterior reversible encephalopathy syndrome
• angiotensin-converting enzyme inhibitor
• renal failure

Conflict of interest

None.