CINRYZE

Country: Australia

Language: English

Source: Department of Health (Therapeutic Goods Administration)

Active ingredient:

C1 esterase inhibitor

Available from:

Viropharma Pty Ltd

Class:

Medicine Registered

Patient Information leaflet

WHAT IS IN THIS LEAFLET
READ ALL OF THIS LEAFLET CAREFULLY BEFORE YOU 
START TAKING CINRYZE. IT PROVIDES 
INFORMATION ON:
•  WHAT CINRYZE IS USED FOR
•  HOW CINRYZE IS GIVEN
•  WHILE YOU ARE HAVING CINRYZE
•  PRODUCT DESCRIPTION
•  Keep this leaflet. You may need to read it again. 
It does not contain all the information about 
CINRYZE.
•  If you have any further questions, ask your doctor 
or pharmacist.
•  All medicines have benefits and risks. Your doctor 
has weighed the benefits that CINRYZE will have for 
you against the risks.
•  This medicine has been prescribed for you. Do not 
pass it on to others. It may harm them, even if their 
symptoms are the same as yours.
•  If you get any side effects that worry you, or if you 
notice any side effects not listed in this leaflet, 
please tell your doctor or pharmacist.
•  IF YOU HAVE ANY CONCERNS ABOUT TAKING THIS 
MEDICINE, ASK YOUR DOCTOR. FOLLOW YOUR 
DOCTOR'S ADVICE EVEN IF IT IS DIFFERENT FROM 
WHAT IS IN THIS LEAFLET.
•  The information in this leaflet is subject to change.
•  PLEASE CHECK WITH YOUR DOCTOR WHETHER 
THERE IS ANY NEW INFORMATION ABOUT THIS 
MEDICINE THAT YOU SHOULD KNOW SINCE YOU 
WERE LAST TREATED WITH THIS MEDICINE.
WHAT CINRYZE IS USED FOR
CINRYZE contains the human protein C1 esterase 
inhibitor as the active substance. 
•  C1 esterase inhibitor is a naturally occurring 
human protein that is normally present in blood. If 
you have a low amount of C1 esterase inhibitor in 
your blood or your C1 esterase inhibitor is not 
working properly, this can lead to swelling attacks 
(called angioedema).  
Symptoms of hereditary angioedema (HAE) may 
include:
•  stomach pains and 
•  swelling of the: 
- hands and feet
- face, eyelids, lips or tongue
- voice-box (larynx), which may make breathing          
  difficult  
- genitals
HOW CINRYZE WORKS
In adults and adolescents, CINRYZE can raise the 
amount of C1 esterase inhibitor in blood and either 
prevent these swel

Summary of Product characteristics

DESCRIPTION  
CINRYZE as a single-use powder vial contains 500 Units of C1 
esterase inhibitor (human) purified from the plasma of human 
donors. One Unit is equivalent to the mean quantity of C1 esterase 
inhibitor present in 1 ml of normal human plasma.  
After reconstitution, a single dose is 1000 Units/10 ml with a 
concentration of 100 Units/ml.
CINRYZE, as a white powder, contains the following excipients.
Sodium chloride (21 mg)  
Sucrose (103 mg)
Sodium citrate (13 mg)
Valine (10 mg)
Alanine (6 mg)
Threonine (23 mg)
PHARMACOLOGY
Pharmacotherapeutic group: Drugs used in hereditary 
angioedema, C1 inhibitor, plasma derived, ATC code: B06AC01.
_MECHANISM OF ACTION_
C1 esterase inhibitor is a member of the serine protease inhibitor, 
or serpin, superfamily of proteins. The main function of serpins is 
to regulate the activity of serine proteases. C1 esterase inhibitor
is 
a single chain glycoprotein found in plasma which, in its mature 
state, consists of 478 amino acids with an apparent molecular 
weight of 105 kD.  
C1 esterase inhibitor inhibits the complement system by binding 
C1r and C1s, two of the active enzyme subunits of the first 
component of the complement system (C1) in the classical 
pathway, as well as to mannose-binding lectin-associated serine 
proteases in the lectin pathway. The primary substrate of the 
activated C1 enzyme is C4; uninhibited C1 results in diminished 
C4 levels. C1 is the most important inhibitor of contact activation 
and regulates the contact system and the intrinsic coagulation 
pathway by binding to and inactivating kallikrein and factor XIIa.  
Because these pathways are part of enzyme amplification 
cascades, without C1 esterase inhibitor, spontaneous or 
trigger-induced activation of these pathways can lead to 
unopposed activation and swelling.
_PHARMACODYNAMIC EFFECTS_
In a study of acute treatment of hereditary angioedema (HAE), 
intravenous administration of CINRYZE resulted in a significant 
increase in systemic levels of antigenic