title: Renal recovery with eculizumab in atypical hemolytic uremic syndrome following prolonged dialysis.
abstract: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy (TMA) which encompasses hemolytic anemia, thrombocytopenia, and organ impairment. Around 10% of cases are atypical HUS (aHUS), a rare disease with poor outcomes caused by uncontrolled activation of the alternative complement pathway. This case describes a young woman with clinical manifestations compatible with TMA during childhood and adolescence who was formally diagnosed with aHUS at the age of 21. She was managed with intensive plasma exchange and hemodialysis, which failed to improve her severe acute kidney injury and other hematological manifestations of aHUS. This was further compounded by several episodes of flash pulmonary edema and the posterior reversible encephalopathy syndrome (PRES). Treatment with the monoclonal anti-C5 inhibitor, eculizumab, improved all hematological parameters with almost full renal recovery following 3.5 months of dialysis. So far, long-term use of eculizumab (> 11 months) continues to be effective and without complication. Our case illustrates the difficulty but importance of early consideration of aHUS in patients presenting with TMA. More importantly, we highlight that near-normal renal recovery may be attained with eculizumab in adults even after a long dependence on dialysis - an observation that has not been reported in the literature so far.
authors.forename: Hannah
authors.initials: H
authors.lastname: Povey
authors.forename: Rahul
authors.initials: R
authors.lastname: Vundru
authors.forename: Naushad
authors.initials: N
authors.lastname: Junglee
authors.forename: Mahdi
authors.initials: M
authors.lastname: Jibani
mesh_terms: D058186:Acute Kidney Injury / Q000628:therapy*; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome / Q000628:therapy*; D003170:Complement Pathway, Alternative; D005260:Female; D006801:Humans; D010951:Plasma Exchange; D054038:Posterior Leukoencephalopathy Syndrome; D006435:Renal Dialysis; D055815:Young Adult
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab