Atypical HUS (Compliment Mediated HUS)

Presentation :

• Primarily occurs in children + young adults
• Typically comes after triggering event: GI illness, viral illness, pregnancy, vaccination
• Many present with renal failure and require renal replacement therapy on presentation (80% of children, 60% of adults)

Pathophysiology :

Diagnostic Testing:

• Exclude other etiologies of TMA

Treatment :

• Eculizumab
  • Monoclonal against C5, preventing cleavage to C5a/C5b which would eventually become part of membrane attack complex (MAC)
  • Early initiation associated with reduced HD rates, ICU time, lower hospitalization costs
  • Typically dosed every 1-2 weeks
• Ravulizumab
  • Long acting version of Eculizumab
  • From 12d to 52d
  • Typically dosed every 8 weeks

Prognosis:

References: