Plasma Infusion versus plasma exchange as first line therapy in atypical hemolytic uremic syndrome: A single centre experience

Abstract

Plasma exchange (PEX) remains the standard therapy for the management of atypical hemolytic-uremic syndrome (aHUS) in regions where eculizumab is not available. Where even PEX is not feasible or afforded, patients are managed with plasma infusion (PI). This single-center, retrospective study compares the efficacy of PI with PEX and examined for predictors of renal recovery in pediatric patients with aHUS admitted at a single center in a developing country from January 2005 to June 2017. Endpoints for comparison were time to hematological remission, duration of hospitalization, rates of complete and partial renal recovery, time to progression to chronic kidney disease Stage 5, and occurrence of relapses. Of 53 children presenting at 6.3 ± 3.9 years of age, 24 and 22 patients were managed with PI and PEX, respectively. The latter group of patients was sicker at presentation and included 13 children with antibodies to complement factor H. All patients achieved hematological remission, including four patients refractory to PI who were switched to PEX. Patients managed on PI were discharged from hospital earlier than those on PEX and were more often with complete renal recovery (55% vs. 27%). At 1.5 (0.5–72) months of follow-up, similar proportions of 31 patients managed with PI and PEX showed complete and partial renal recovery and had comparable rates of relapse and disease progression. Presentation with acute kidney injury Stage 3 and edema were independent predictors of incomplete renal recovery at discharge. Both PEX and PI are associated with satisfactory short-term outcomes in patients with aHUS lacking access to eculizumab, and PEX might be preferred over PI in patients with severe disease and those with anticomplement factor H antibodies.