• Keep this leaflet. You may need to read it again.
• If you have any further questions, ask your doctor or healthcare professional.
• If you get any side effects, talk to your doctor or healthcare professional. This includes any possible side effects not listed in this leaflet.

1. What Privigen is and what it is used for
2. What you need to know before you are given Privigen
3. How to use Privigen
4. Possible side effects
5. How to store Privigen
6. Contents of the pack and other information

Privigen belongs to the class of medicines called human normal immunoglobulins.

Immunoglobulins are also known as antibodies and are blood proteins that help your body to fight infections.

Privigen contains immunoglobulins that have been prepared from the blood of healthy people. The medicine works in exactly the same way as the immunoglobulins naturally present in human blood of healthy people.

Privigen is used for the treatment of adults and children (0-18 years) in the following situations:

A) To increase abnormally low immunoglobulin levels in your blood to normal levels (replacement therapy).

1. Patients who are born with a reduced ability or inability to produce immunoglobulins (primary immunodeficiencies (PID)).
2. Patients with an acquired immunodeficiency (SID) who suffer from severe or recurrent infections, ineffective antimicrobial treatment and either proven specific antibody failure or serum IgG level of <4 g/l.

B) To treat certain inflammatory disorders (immunomodulation). There are five groups:

1. Patients who do not have enough blood platelets (primary immune thrombocytopenia (ITP)) and who are at high risk of bleeding or will have surgery in the near future.
2. Patients with Guillain-Barré syndrome. This is an acute disease that is characterised by inflammation of the peripheral nerves that causes severe muscle weakness mainly in the legs and upper limbs.
3. Patients with Kawasaki disease. This is an acute disease that primarily affects young children. It is characterised by inflammation of blood vessels throughout the body.
4. Patients with chronic inflammatory demyelinating polyneuropathy (CIDP). This is a chronic disease that is characterised by inflammation of the peripheral nerves that causes muscle weakness and/or numbness mainly in the legs and upper limbs.
5. Patients with multifocal motor neuropathy (MMN). This is a slowly progressive disease of the motor nerves with weakness of arms and legs.

Which circumstances increase the risk of having side effects?

• Tell your doctor or healthcare professional prior to treatment if any of the circumstances listed below applies to you:

• You receive this medicine in high doses either on 1 day or over several days and you have a blood group A, B or AB and/or you have an underlying inflammatory condition. In these circumstances, it has been commonly reported that immunoglobulins increase the risk of breakdown of red blood cells (haemolysis).
• You are overweight, are elderly, have diabetes, have been bedridden for a long time, have high blood pressure, have low blood volume (hypovolaemia), have problems with your blood vessels (vascular diseases), have an increased tendency for blood clotting (thrombophilia or thrombotic episodes) or have a disease or a condition which causes your blood to thicken (hyperviscous blood). In these circumstances, immunoglobulins may increase the risk of heart attack (cardiac infarction), stroke, blood clots in the lung (lung embolism), or blockage of a blood vessel in the leg, although only very rarely.
• You are diabetic. Although Privigen does not contain sugar, it may be diluted with a special sugar solution (5% glucose), which could affect your blood sugar level.
• You have or had previously problems with your kidneys or take medicinal products that may harm your kidneys (nephrotoxic medicinal products). In these circumstances, immunoglobulins may increase the risk of serious rapid loss of kidney function (acute renal failure) although only very rarely. Loss of kidney function with fatal outcome has occurred in isolated haemolysis-related cases.

What kind of monitoring is required during the infusion?

For your personal safety, treatment with Privigen will take place under the supervision of your doctor or healthcare professional. You will usually be observed during the whole infusion and for at least 20 minutes thereafter. In certain circumstances, special precautions may be necessary. Examples of such circumstances are:

• you are receiving Privigen at a high infusion rate or
• you are receiving Privigen for the first time or after a long break in treatment (e.g. several months).

In these cases you will be closely observed during the whole infusion and for at least 1 hour afterwards.

When may slowing or stopping the infusion be required?

• You may be allergic (hypersensitive) to immunoglobulins without knowing it. However, true allergic reactions are rare. They may occur even if you have previously received human immunoglobulins and had tolerated them well. It may happen particularly if you have developed antibodies against immunoglobulins of the type IgA. In these rare cases allergic reactions such as a sudden fall in blood pressure or shock may occur (see also section 4 “Possible side effects”).
• In very rare cases, transfusion-related acute lung injury (TRALI) can occur after receiving immunoglobulins. This will lead to non-heart related accumulation of fluid in the air spaces of the lungs (non-cardiogenic pulmonary oedema). You will recognize TRALI by severe difficulty in breathing (respiratory distress), bluish skin (cyanosis), abnormally low level of oxygen in the blood (hypoxia), decrease in blood pressure (hypotension) and increased body temperature (fever). Symptoms typically appear during or within 6 hours after receiving treatment.
  • Tell your doctor or healthcare professional immediately if you notice such reactions during the infusion of Privigen. He or she will decide whether to decrease the infusion rate or to stop the infusion completely.

Blood tests

• Tell your doctor about your treatment with Privigen prior to having any blood tests.

After receiving Privigen, the results of certain blood tests (serological tests) may be impaired for a certain time.

Information on safety with respect to infections

Privigen is made from human blood plasma (this is the liquid part of the blood).

When medicines are made from human blood or plasma, certain measures are put in place to prevent infections being passed on to patients. These include

• careful selection of blood and plasma donors to make sure those at risk of carrying infections are excluded
• the testing of each donation and pools of plasma for signs of virus/infections.
• the inclusion of steps in the processing of the blood or plasma that can inactivate or remove viruses.

Despite these measures, when medicines prepared from human blood or plasma are administered, the possibility of passing on infection cannot be totally excluded. This also applies to any unknown or emerging viruses and other types of infections.

The measures taken are considered effective for enveloped viruses such as human immunodeficiency virus (HIV), hepatitis B virus and hepatitis C virus, and for the non-enveloped hepatitis A virus and parvovirus B19.

Immunoglobulins have not been associated with hepatitis A or parvovirus B19 infections, possibly because antibodies against these infections, which are contained in the product, are protective.

• It is strongly recommended that every time you are given a dose of Privigen the name and batch number of the product are recorded in order to maintain a record of the batches used.

• Tell your doctor or healthcare professional if you are using, have recently used or might use any other medicines.

The concomitant use of medicines that increase the excretion of water from your body (loop diuretics) should be avoided during treatment with Privigen. Your doctor will decide whether you should use or continue treatment with loop diuretics.

Vaccinations

• Tell your vaccinating doctor prior to a vaccination about your treatment with Privigen.

After receiving Privigen, the efficacy of certain vaccinations may be impaired. Affected are vaccinations with live attenuated virus vaccines such as vaccinations against measles, mumps, rubella and varicella. Such vaccinations should be postponed for at least 3 months after the last infusion of Privigen. In the case of measles vaccinations the impairment may persist for up to 1 year. Therefore, your vaccinating doctor should check the effectiveness of the measles vaccination.

• Tell your doctor or healthcare professional if you are pregnant, plan to become pregnant or are breast-feeding. Your doctor will decide whether you can receive Privigen during your pregnancy or while you are breast-feeding.

Medicines containing antibodies have been used in pregnant and breast-feeding women. Long-term experience has shown that no harmful effects during the course of the pregnancy or to the newborn are to be expected.

If you receive Privigen while you are breast-feeding the antibodies in this medicine will also be found in the breast milk. Thus, also your baby can receive the protective antibodies.

Patients may experience effects, such as dizziness or nausea, during treatment with Privigen that might affect the ability to drive and use machines. If this happens, you should not drive or use machines until these effects have disappeared.

You must not take it if you suffer from hyperprolinaemia (see also section 2 “What you need to know before you are given Privigen”).

• Tell your doctor prior to treatment.

This medicine contains less than 2.3 mg sodium (main component of cooking/table salt) in 100 ml. This is equivalent to 0.12% of the recommended maximum daily dietary intake of sodium for an adult.

Overdose is very unlikely to occur because Privigen is usually administered under medical supervision. If, in spite of this, you receive more Privigen than you should, your blood may become too thick (hyperviscous), which might increase the risk of developing blood clots.

This may happen particularly if you are a patient at risk, for example if you are elderly or if you suffer from a heart or kidney disease. Tell your doctor if you are known to have medical problems.

If you get any side effects, talk to your doctor or healthcare professional. This includes any possible side effects not listed in this leaflet. You can also report side effects directly via the UK Yellow Card Scheme at www.mhra.gov.uk/yellowcard or search for MHRA Yellow Card in the Google Play or Apple App Store.

By reporting side effects, you can help provide more information on the safety of this medicine.

• The active substance is human normal immunoglobulin (antibodies of the type IgG).
  Privigen contains 100 mg/ml (10%) human protein of which at least 98% is IgG.
  The approximate percentage of IgG subclasses is as follows:
  IgG₁ ................... 69%
  IgG₂ ................... 26%
  IgG₃ ..................... 3%
  IgG₄ ..................... 2%
  This medicine contains trace amounts of IgA (not more than 25 micrograms/ml).
• The other ingredients (excipients) are the amino acid proline, water for injections, and hydrochloric acid or sodium hydroxide (for pH adjustment).

Privigen is presented as a solution for infusion.

The solution is clear or slightly opalescent and colourless to pale-yellow.

Pack sizes:

1 vial (2.5 g/25 ml, 5 g/50 ml, 10 g/100 ml, 20 g/200 ml) or 40 g/400ml,

3 vials (10 g/100 ml or 20 g/200 ml).

Not all pack sizes may be marketed.