@article{discovery10073189,
            note = {This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.},
          volume = {186},
           pages = {113--124},
          number = {1},
           month = {July},
         journal = {British Journal of Haematology},
           title = {Atypical haemolytic uraemic syndrome in the eculizumab era: presentation, response to treatment and evaluation of an eculizumab withdrawal strategy},
            year = {2019},
        keywords = {aHUS, TMA, atypical haemolytic uraemic syndrome, eculizumab, withdrawal},
            issn = {0007-1048},
        abstract = {The complement inhibitor, eculizumab, has revolutionised the management of atypical haemolytic uraemic syndrome (aHUS), although the optimum treatment duration is debated. Twenty-two cases of acute aHUS managed with eculizumab were retrospectively reviewed, including outcomes after eculizumab withdrawal. Although 41\% had an associated complement genetic abnormality, mutation status did not affect severity of clinical presentation. Sixty-four percent required renal replacement acutely, with a high incidence of nephrotic range proteinuria (47\%). Eculizumab followed a median of 6�days of plasma exchange. After a median duration of therapy of 11�weeks (range 1-227), haematological recovery was seen in 100\%, while 81\% achieved at least partial renal recovery (median increase in estimated glomerular filtration rate (eGFR) 49�ml/min/1.73�m2 ). At median duration of follow-up of 85�weeks (range 4-255), 54.5\% had eGFR�{$\ge$}�60�ml/min/1.73�m2 , 27\% had CKD, 14\% were on dialysis, and 4.5\% had died. Eculizumab was withdrawn in 59\% (13/22) cases following complete haematological and renal recovery. Three of these 13 patients (23\%) subsequently relapsed, with defined triggers in 2/3, but all made a full recovery with rapid resumption of eculizumab. There was a significant association between higher presenting creatinine and poorer renal outcomes. A strategy of eculizumab withdrawal in selected cases is both safe and cost effective.},
          author = {Neave, L and Gale, DP and Cheesman, S and Shah, R and Scully, M},
             url = {https://doi.org/10.1111/bjh.15899}
}