Gianluigi Ardissino, Donata Cresseri, Maria Cristina Mancuso, Valentina Capone, Luigi Porcaro, Valeria Amico, Marianna Tangredi, Elena Grovetti, Samantha Griffini, Giuseppe Castellano, Giovanni Montini, Dario Consonni, Massimo Cugno
BACKGROUND: Atypical-hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy often due to uncontrolled complement activation, characterized by high risk of end-stage kidney disease (ESKD). Eculizumab has improved the outcome, however, its efficacy varies among patients and its discontinuation is debated. METHODS: To identify characteristics associated with treatment response, we analyzed 244 aHUS patients referred to our center. Patients were classified according to the presence/absence of complement abnormalities and/or triggers at onset in 4 categories: (1) primary (complement abnormality without trigger), (2) secondary (trigger without complement abnormality), (3) combined (trigger and complement abnormality), (4) idiopathic (no trigger, no complement abnormality)...
January 27, 2024: Journal of Nephrology