Atypical hemolytic-uremic syndrome

Pathophysiology

• Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity

Investigations

• Diagnosis with genetic mutation analysis of complement regulatory proteins (CFH, CFI, MCP, C3, CFB, THBD) and anti-CFH antibodies

Management

• Often unable to distinguish from TTP, so plasma exchange should be initiated promptly
• If no improvement on PLEX and there is significant renal involvement, consider aHUS-specific treatment
• Eculizumab