Early relapse rate determines further relapse risk: results of a 5-year follow-up study on pediatric CFH-Ab HUS

• DOI: 10.1007/s00467-020-04751-9
• Publication Date: 2020-10-07T03:41:50Z
• Authors: Johannes Hofer; Magdalena Riedl K...; Markus Perl; Thomas Giner; Alejandra Rosales; Gerard Cortina; Siegfied Waldegger; Therese Jungraith...; Reinhard Würzner
• Keywords: Nephrology

The complement factor H antibody (CFH-Ab)-associated hemolytic uremic syndrome (HUS) forms a distinct subgroup within the complement-mediated HUS disease spectrum. autoimmune nature of this implies potential benefit targeted immunosuppressive therapy. Data on long-term outcome are scarce.This observational study evaluates clinical 19 pediatric CFH-Ab patients from onset until their 5-year follow-up.All but one relapse occurred during first 2 years, and who had no 6 months were relapse-free end observation period. Kidney function at determines kidney function: all individuals with normal after 5 reduced impaired last follow-up. Level titer was not correlated higher risk recurrences or worse years. Resolution titers years common.CFH-Ab have varied overall course. Early relapses common, making close surveillance essential, regardless initial titer.