Can eculizumab be discontinued in aHUS? Case report and review of the literature

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Sahutoglu T., Basturk T., Sakaci T., Koc Y., Ahbap E., Sevinc M., ...More

MEDICINE, vol.95, no.31, 2016 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 95 Issue: 31
  • Publication Date: 2016
  • Doi Number: 10.1097/md.0000000000004330
  • Journal Name: MEDICINE
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Keywords: aHUS, eculizumab, mutation of complement factors, HEMOLYTIC-UREMIC SYNDROME, COMPLEMENT ACTIVATION, MAINTENANCE TREATMENT, THERAPY, REMISSION
  • Recep Tayyip Erdoğan University Affiliated: Yes

Abstract

Background: The management of atypical hemolytic uremic syndrome (aHUS) has evolved into better control of thrombotic microangiopathy (TMA) and recovery of renal functions since the recent introduction of the terminal complement cascade blocker, eculizumab, into clinical use. Better characterization of genotype phenotype relations has become possible with genetic and clinical studies. However, these advances brought up some important issues, such as the possibility and timing of discontinuation of eculizumab and strategy of follow-up that need to be enlightened.