Successful treatment of anti-factor H antibody-associated atypical hemolytic uremic syndrome

• Main Authors: Tomoo Kise, Shigeru Fukuyama, Masatsugu Uehara
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2020-01-01
• Series: Indian Journal of Nephrology
• Subjects: anti-cfh antibody; ahus; child; eculizumab
• Online Access: http://www.indianjnephrol.org/article.asp?issn=0971-4065;year=2020;volume=30;issue=1;spage=35;epage=38;aulast=Kise

Anti-complement factor H (CFH) autoantibody (Ab)-associated atypical hemolytic uremic syndrome (aHUS) has a poor prognosis in terms of frequent relapses. Although eculizumab is an effective treatment for this type of aHUS, the method of eculizumab discontinuation is not yet established. Herein, we report a case of anti-CFH Ab-associated aHUS in a 6-year-old boy. Eculizumab induction therapy following plasma exchange improved his condition. After 14 months, eculizumab was discontinued because of meningococcal bacteremia. After 6 months of eculizumab cessation, prednisolone (20 mg/alternate days) and mycophenolate mofetil (500 mg/day) were initiated. There were no relapses or increases in anti-CFH Ab titers for 26 months after treatment initiation. We believe that eculizumab induction therapy, following plasma exchange and maintenance therapy with immunosuppressants after eculizumab discontinuation are effective treatments for anti-CFH Ab-related aHUS.