What is primary immunodeficiency (PI)?

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 400 different PIs currently recognized by the International Union of Immunological Societies—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:

  • Skin
  • Sinuses
  • Throat
  • Ears
  • Lungs
  • Brain or spinal cord
  • Urinary or intestinal tract
PI is an immune system disorder

Treating PI with Ig therapy

Many patients with PI are living healthy lives, thanks to immunoglobulin (Ig) replacement therapy, which replaces the antibodies they are missing. Since half of the antibodies they receive in the Ig infusions are metabolized over 3 to 4 weeks, repeat doses are required at regular intervals. Ig replacement is usually necessary for the patient's whole life.4

There are 2 main types of administration for Ig therapy to treat PI: intravenous Ig (IVIg) therapy, like Privigen, and subcutaneous.

Below are details on IVIg5:

IVIg therapy
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

Delaying treatment can lead to irreversible damage

Hear from PI experts Carla Duff, ARNP,* and Mark Stein, MD, about the consequences of delaying diagnosis and treatment of PI.

*Carla Duff, ARNP, is currently an employee of CSL Behring. When this video was produced, Ms. Duff was a pediatric nurse practitioner at the Johns Hopkins All Children’s Hospital in St. Petersburg, Florida.

Patients may prefer Privigen IVIg therapy

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.

Privigen can be administered once every 3-4 weeks

If you think your patient can benefit from SCIg therapy, learn about Hizentra®   (Immune Globulin Subcutaneous [Human] 20% Liquid

What to look for in IVIg products

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:

  • Sugar and sodium content that may cause adverse reactions in certain patient types6
  • Storage requirements (may require constant refrigeration) and shelf life7
  • Ready-to-use liquid preparation or lyophilized powder that requires reconstitution7
  • Manufacturing processes6
  • Dimer, fragment, and aggregate content7
  • Stabilizer, IgA content, and osmolality6
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What is chronic inflammatory demyelinating polyneuropathy (CIDP)?

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

Symptoms of CIDP may include8,9:

  • Tingling or numbness, beginning in the toes and fingers
  • Weakness of the arms or legs
  • Loss of reflexes
CIDP patient taking a photo

Treating CIDP with Ig therapy

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, Ig therapies are the only treatments with FDA, Canadian, and European approval for CIDP.8

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If you think your patient can benefit from SCIg therapy, learn about Hizentra®   (Immune Globulin Subcutaneous [Human] 20% Liquid

What is immune thrombocytopenic purpura (ITP)?

Immune thrombocytopenic purpura (ITP) is an immune system bleeding disorder due to low platelet counts. Patients with ITP often exhibit purpura (red, purple, or brownish-yellow spots) in the skin and mucous membranes and petechiae (red or purple dots) under 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 stimulus11; it may occur in isolation (primary) or in association with other diseases (secondary).12

Chronic ITP is an immune system bleeding disorder

ITP also may cause life-threatening bleeding in the brain or other organs.10 The American Society of Hematology, citing work of an international working group, defines 3 forms of ITP.12

Newly diagnosed : Diagnosis to 3 months Persistent : 3-12 months from diagnosis Chronic : Lasting more than 12 months from diagnosis

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.13 See the FAQs for additional information about ITP.

Treating chronic ITP with Ig therapy

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.13 Platelet transfusions may be needed in severe cases.12,14

Privigen provides proven efficacy

See the clinical trial results for Privigen in chronic ITP

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