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Abstract

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

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.


Author(s):

Johannes Hofer, Hannah-Sophie Arbeiter, Mihály Józsi, Thomas Giner, Diana Dobiliene, Jurate Masalskiene, Jonas Surkus and Sarunas Rudaitis



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