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. 2018 Sep 10;8(5):122–141. doi: 10.5500/wjt.v8.i5.122

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©The Author(s) 2018. Published by Baishideng Publishing Group Inc. All rights reserved.

This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial.

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Recommendations for cessation of treatment with complement inhibitors. There are no prospective controlled studies in patients with atypical hemolytic uremic syndrome (aHUS) to define criteria for discontinuation of eculizumab therapy. This flow diagram is based on expert opinion[176-178]. Discontinuation can be considered on a case-by-case basis in patients after at least 6-12 mo of treatment and at least 3 mo of normalization (or stabilization in the case of residual CKD) of kidney function. Earlier cessation (at 3 mo) may be considered in patients (especially children) with pathogenic variants in MCP if there has been rapid remission and recovery of renal function. Patients on dialysis, eculizumab should be maintained for at least 4 to 6 mo before discontinuation. In this setting, assessment of fibrotic changes in kidney biopsy may be helpful. In transplant patients, especially patients who have lost previous allografts, discontinuation is not recommended. Adapted from: Goodship et al[58]. aHUS: Atypical hemolytic uremic syndrome.