Eculizumab (Soliris)

Indications

  • Atypical Hemolytic Uremic Syndrome (see Hemolytic-Uremic Syndrome, [[Hemolytic-Uremic Syndrome]])
  • Paroxysmal Nocturnal Hemoglobinuria (PNH) (see Paroxysmal Nocturnal Hemoglobinuria, [[Paroxysmal Nocturnal Hemoglobinuria]])
    • Licensed to treat PNH in 2007
    • Demonstrated to decrease need for PRBC transfusions in only 50% of cases (presumably due to extravascular hemolysis of cells opsonized by C3 fragments)
    • Extremely expensive (approximate $400k per year)
    • Requires lifelong therapy q14 days

Pharmacology

  • Human Monoclonal Antibody Against Complement C5
    • Inhibits the cleavage of C5 to C5a and C5b by C5 convertase -> inhibits the generation of the terminal complement complex C5b-9

Administration

  • IV Infusion
    • 600 mg qweek for the first 4 wks
    • 900 mg for the fifth dose 1 wk later
    • 900 mg q2 wks thereafter

Adverse Effects

Infectious Adverse Effects

  • Infection with Encapsulated Bacteria (see Bacteria and Fungi, [[Bacteria + Fungi]])
  • Nasopharyngitis
  • Upper Respiratory Infection

Gastroenterologic Adverse Effects

Other Adverse Effects

  • Back Pain
  • Cough (see Cough, [[Cough]])
  • Fatigue
  • Headache (see Headache, [[Headache]])
  • Hypertension (see Hypertension, [[Hypertension]])

References

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