Atypical hemolytic-uremic syndrome
From IDWiki
Background
Pathophysiology
- Congenital defect leading to dysregulation of the alternative complement pathway, which leads to increased complement activity
Diagnosis
- 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 an aHUS-specific treatment
- Eculizumab to inhibit complement, ideally with full meningococcal vaccination beforehand
