A Case of CFH-Ab Hus Responsive to Plasmapheresis and with Sustained Remission after Initiation of Immunosuppressive Therapy Highlighting the Common Pit Falls of a Complicated Disease

Johannes Hofer, Hannah-Sophie

Abstract

Background: Complement factor H antibody (CFH-Ab) associated hemolytic uremic syndrome (HUS) is different from the genetically defined aHUS forms, especially concerning diagnosis and treatment. Case: We report a case of aHUS responsive to plasmapheresis with a slightly delayed detection of CFHAb after first disease recurrence. The initiation of immunosuppressive induction and maintenance therapy led to a sustained complete remission. Conclusion: This case of CFH-Ab HUS responsive to PP and remission after initiation of IS supports the recently published guidelines on therapy of CFH-Ab aHUS and highlights the importance of a fast diagnosis of this rare disease entity.