Volume 85, Issue 5, Pages (May 2014)

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Volume 85, Issue 5, Pages 1151-1160 (May 2014) Prompt plasma exchanges and immunosuppressive treatment improves the outcomes of anti-factor H autoantibody-associated hemolytic uremic syndrome in children  Aditi Sinha, Ashima Gulati, Savita Saini, Caroline Blanc, Aarti Gupta, Bahadur Singh Gurjar, Himanshi Saini, Shambhuprasad T. Kotresh, Uma Ali, Divya Bhatia, Alpana Ohri, Manish Kumar, Indira Agarwal, Sanjeev Gulati, Kanav Anand, M. Vijayakumar, Rajiv Sinha, Sidharth Sethi, Maud Salmona, Anna George, Vineeta Bal, Geetika Singh, Amit K. Dinda, Pankaj Hari, Satyajit Rath, Marie-Agnes Dragon-Durey, Arvind Bagga  Kidney International  Volume 85, Issue 5, Pages 1151-1160 (May 2014) DOI: 10.1038/ki.2013.373 Copyright © 2014 International Society of Nephrology Terms and Conditions

Figure 1 Anti-complement factor H antibody titers (mean±s.e.m.) in relation to therapy and outcome. (a) Antibody titers, repeated in 54 patients at a median of 32 (interquartile range 11–84) days of plasma exchanges, were significantly lower (P<0.0001) compared with those at onset. (b) Following therapy with intravenous immunoglobulin (IVIG) and plasma exchange (n=21), antibody titers declined significantly (P<0.0001) at a median of 15 (interquartile range 9–28) days. Most patients in both groups were also receiving immunosuppressive medications. (c) Serial samples, in 10 patients, showing that titers declined at remission (P=0.0008) and increased during relapse (P=0.006). (d) Antibody titers were significantly higher at disease presentation in patients with adverse outcome at 3 months (P=0.010). Kidney International 2014 85, 1151-1160DOI: (10.1038/ki.2013.373) Copyright © 2014 International Society of Nephrology Terms and Conditions

Figure 2 Probability of renal survival in patients with anti-complement factor H antibody-associated hemolytic uremic syndrome. Patients who received combined therapy with plasma exchanges and induction immunosuppression showed 83.0% survival at 6 months, 75.6% at 12 months, and 71.2% at last follow-up (interrupted line). Corresponding renal survival in patients not receiving combined therapy (continuous line) was 46.1, 41.5, and 33.2% (log rank P<0.0001). Kidney International 2014 85, 1151-1160DOI: (10.1038/ki.2013.373) Copyright © 2014 International Society of Nephrology Terms and Conditions

Figure 3 Probability of disease relapses with respect to maintenance immunosuppression. Relapse-free survival was 95.3% at 6 months, 92.3% at 12 months, and 87.2% during follow-up in patients receiving maintenance therapy (interrupted line), compared with 76.8, 69.1, and 46.1% in patients not receiving such therapy (continuous line) (log rank P=0.010). Kidney International 2014 85, 1151-1160DOI: (10.1038/ki.2013.373) Copyright © 2014 International Society of Nephrology Terms and Conditions