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BERINERT (human C1 esterase inhibitor)

RARE DISEASE - New indication/Update
Opinions on drugs - Posted on Feb 08 2019

Reason for request

Re-assessment of the actual benefit and of the improvement in actual benefit

High clinical benefit in their respective indications, but no demonstrated clinical advantage, with the exception of BERINERT in adults, adolescents and children, and FIRAZYR in adults, representing a moderate clinical added value for the management of acute hereditary angioedema attacks.

 

  • BERINERT, CINRYZE, FIRAZYR and RUCONEST have been granted an MA for the treatment of acute hereditary angioedema attacks (HAE) and/or for short-term and/or long-term prevention in children, adolescents and adults, according to their respective MAs.

 

 

Treatment of acute attacks

Short-term prevention

Long-term prevention

CINRYZE

(children, adolescents, adults)

(children, adolescents, adults)

(children, adolescents, adults)

BERINERT

(paediatrics, adults)

(paediatrics, adults)

-

FIRAZYR

(children, adolescents, adults)

-

-

RUCONEST

(adolescents, adults)

-

-

 

  • Subcutaneous administration of FIRAZYR, with self-administration by the patient possible after learning, presents a benefit relative to IV administration, particularly when used in an emergency context. Its short plasma half-life may require a second injection. BERINERT and CINRYZE, administered IV, have longer half-lives, respectively of 36 and 56 h, than those of FIRAZYR and RUCONEST.

  • RUCONEST may contain traces of rabbit protein, thus requiring precautions prior to its use, though it is administered in an emergency context.

 

 


Clinical Benefit

Substantial

-


Clinical Added Value

moderate

-

no clinical added value

Therapeutic use

-