Please use this identifier to cite or link to this item: http://hdl.handle.net/123456789/827
Title: Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children
Authors: Bagga, Arvind
Sinha, Aditi
Gulati, Ashima
Saini, Savita
Blanc, Caroline
Gupta, Aarti
Gurjar, Bahadur Singh
Saini, Himanshi
Kotresh, Shambhuprasad T
Ali, Uma
Bhatia, Divya
Ohri, Alpana
Kumar, Manish
Agarwal, Indira
Gulati, Sanjeev
Anand, Kanav
Vijayakumar, M
Sinha, Rajiv
Sethi, Sidharth
Salmona, Maud
George, Anna
Bal, Vineeta
Singh, Geetika
Dinda, Amit K
Hari, Pankaj
Rath, Satyajit
Dragon-Durey, Marie-Agnes
Keywords: Nephrology
Issue Date: May-2014
Publisher: International Society of Nephrology
Abstract: Antibodies to complement factor H are an uncommon cause of hemolytic uremic syndrome (HUS). Information on clinical features and outcomes in children is limited. In order to explore this we studied a multicenter cohort of 138 Indian children with anti-complement factor H antibody associated HUS, constituting 56% of patients with HUS. Antibody titers were high (mean 7054 AU/ml) and correlated inversely with levels of complement C3, but not complement factor H. Homozygous deletion of the CFHR1 gene was found in 60 of 68 patients. Therapies included dialysis in 119 children, 105 receiving plasma exchanges and 26 intravenous immunoglobulin. Induction immunosuppression consisted of 87 children receiving prednisolone with or without intravenous cyclophosphamide or rituximab. Antibody titers fell significantly following plasma exchanges and increased during relapses. Adverse outcome (stage 4-5 CKD or death) was seen in 36 at 3 months and 41 by last follow up, with relapse in 14 of 122 available children. Significant independent risk factors for adverse outcome were an antibody titer over 8000 AU/ml, low C3 and delay in plasma exchange. Combined plasma exchanges and induction immunosuppression resulted in significantly improved renal survival: one adverse outcome prevented for every 2.6 patients treated. Maintenance immunosuppressive therapy, of prednisolone with either mycophenolate mofetil or azathioprine, significantly reduced the risk of relapses. Thus, prompt use of immunosuppressive agents and plasma exchanges are useful for improving outcomes in pediatric patients with anti-complement factor H-associated HUS.
URI: http://hdl.handle.net/123456789/827
Appears in Collections:Mucosal Immunology, Publications

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