Primary immunodeficiency (PI) diseases occur in patients who have an intrinsic defect in the immune system. The immune system may have impaired function in one or more components, or may be absent altogether. There are more than 300 different PIs currently recognized by the World Health Organization—some are common, others are quite rare. Because patients with PI do not have fully functioning immune systems, they are more susceptible to infection in the2,3:
The overall goal in PI treatment is to restore a patient's immune system with immunoglobulins, allowing him or her to maintain a normal life. Patients with PI may benefit from replacement therapy with immunoglobulin. Since half of the infused antibodies are metabolized over 3 to 4 weeks, repeat doses are required at regular intervals. Immunoglobulin replacement is usually necessary for the patient's whole life.4
|Administered once every 3–4 weeks|
|Reduces the incidence of acute serious bacterial infections (aSBIs)|
|Venous access required|
|Requires trained healthcare personnel, either in the patient's home or in a medical setting|
|Systemic ARs are more common|
|Local site reactions are not expected|
Hear from PI experts Carla Duff, ARNP, and Mark Stein, MD, about the consequences of delayed diagnosis and treatment of PI.
IVIg therapy with Privigen allows patients to receive treatment once every 3–4 weeks by their healthcare team—either at home or in a medical setting—as opposed to more frequent SCIg treatments that may be self-administered.
There are several IVIg products currently available in the United States. While all work to boost the patient's immune system, there are some features and attributes to consider when choosing a preparation to stock or prescribe. These include:
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare disorder of the peripheral nerves characterized by progressive weakness and a loss of sensory and motor function in the legs and arms. CIDP is a neurological disorder with an underlying autoimmune basis: the immune system perceives the nerves' protective myelin as foreign and attacks it. Damaged or removed myelin prevents effective communication in the nervous system, as electric impulses transmitted to and from the brain are disrupted or lost.8,9
Although CIDP is a chronic disease, Ig therapy can limit the damage to peripheral nerves and help improve motor function. IVIg contains naturally occurring antibodies obtained from healthy volunteers and is administered intravenously. Currently, IVIg is the only drug with FDA, Canadian, and European approval for the treatment of CIDP.8
Immune thrombocytopenic purpura (ITP) is an immune system bleeding disorder due to low platelet counts. Patients with ITP often exhibit purpura (purple bruises) on the skin and mucous membranes and petechiae (red or purple dots) on the skin. People with ITP may also experience nosebleeds, gingival bleeding, heavy menstrual bleeding, and hematomas.10 ITP usually does not have an identifiable precipitating stimulus; it may occur in isolation (primary) or in association with other diseases (secondary).11
Privigen is indicated for the treatment of chronic ITP. At the time of the Privigen pivotal trial, chronic ITP was defined as lasting 6 months or longer , and the disease criteria for that study population followed the earlier (>6 months) definition of chronic ITP.12 See the FAQs for additional information about ITP.
Treatment depends on the platelet count and the frequency and amount of bleeding experienced by the patient. Immunoglobulin therapy is used as a treatment to increase platelet counts.12 Platelet transfusions may be needed in severe cases.11,13