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Title of Journal: Pediatr Nephrol

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Abbravation: Pediatric Nephrology

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Publisher

Springer Berlin Heidelberg

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DOI

10.1007/bf01402793

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ISSN

1432-198X

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Successful treatment of DEAPHUS with eculizumab

Authors: Damien Noone Aoife Waters Fred G Pluthero Denis F Geary Michael Kirschfink Peter F Zipfel Christoph Licht
Publish Date: 2013/11/20
Volume: 29, Issue: 5, Pages: 841-851
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Abstract

Deficiency of complement factor Hrelated CFHR proteins and CFH autoantibodypositive hemolytic uremic syndrome DEAPHUS represents a unique subgroup of complementmediated atypical HUS aHUS Autoantibodies to the Cterminus of CFH block CFH surface recognition and mimic mutations found in the genetic form of CFHmediated aHUS CFH autoantibodies are found in 10–15  of aHUS patients and occur—so far unexplained—almost exclusively in the background of CFHR1 or CFHR3/CFHR1 deletionsAs a welldefined role for eculizumab in the treatment of complementmediated aHUS is becoming established its role in DEAPHUS is less conspicuous where a Bcelldepleting and immunosuppressive treatment strategy is being proposed in the literatureWe here show eculizumab to be safe and effective in maintaining a diseasefree state without recurrence in a previously plasmatherapydependent DEAPHUS patient and in another patient in whom although showing a good clinical response to plasma therapy the therapy was hampered by allergic reactions to fresh frozen plasma and contend there is a rationale for the use of eculizumab in concert with an immunosuppressive strategy in the treatment of DEAPHUS Considering the high rate of early relapse the possible coexistence and contribution of both known and unknown complementgene mutations the probable pathogenic role of CFHR1 as a complement alternative pathway CAP regulator the experimental nature of measuring and using antiCFH autoantibodies to guide management and until the positive reports of immunosuppression in addition to plasma therapy are confirmed in prospective studies we feel that a complementdirected therapy should not be neglected in DEAPHUS Serial CFH autoantibody titer testing may become a valuable tool to monitor treatment response and weaning patients off eculizumab may become an option once CFH autoantibody levels are depleted

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