Effect of plasma exchange and immunosuppressive medications on antibody titers and outcome in anti-complement factor H antibody-associated hemolytic uremic syndrome View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2014-09-13

AUTHORS

Priyanka Khandelwal, Aarti Gupta, Aditi Sinha, Savita Saini, Pankaj Hari, Marie-Agnes Dragon Durey, Arvind Bagga

ABSTRACT

BackgroundAnti-complement factor H (anti-CFH) antibody-associated hemolytic uremic syndrome (HUS) is an important cause of acute kidney injury in Indian children. While management comprises plasma exchange and immunosuppression, information on the impact on serial antibody titers and outcomes is limited.MethodsThis retrospective study included 45 patients with anti-CFH-associated HUS who were followed for ≥12 months. Following the initial plasma exchange sessions, patients received prednisolone and either intravenous (IV) cyclophosphamide (n = 31) or IV rituximab (n = 14), followed by maintenance immunosuppression.ResultsThe median anti-CFH antibody titers fell from 3,215.5 [interquartile range (IQR) 1,977.9–8,453.9 to 414.6 (IQR 251.6–1,368.2) AU/ml with plasma exchange therapy (P < 0.0001), and the decline was similar with three, five, or seven plasma exchange sessions (P = 0.08). Serial anti-CFH titers were similar in patients receiving IV cyclophosphamide- and rituximab-based regimens during the 12-month follow-up (P = 0.63). Renal outcomes and relapse frequencies at the 15.4-month follow-up were comparable. Seven patients relapsed 6.5 (IQR 2.2–12.3) months from treatment onset. Patients with relapse had higher antibody titers during remission (P = 0.017). Titers of ≥1,300 AU/ml at 6 months predicted subsequent relapses.ConclusionsOur patients with anti-CFH antibody-associated HUS showed a significant fall in antibody titers following daily plasma exchange sessions. Therapy with cyclophosphamide- or rituximab-based regimens was associated with similar outcomes and a comparable decline in antibody titers. More... »

PAGES

451-457

Identifiers

URI

http://scigraph.springernature.com/pub.10.1007/s00467-014-2948-7

DOI

http://dx.doi.org/10.1007/s00467-014-2948-7

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1045032549

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/25217328


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