Skip to main content
Neutrophil White Blood Cells)

Primary Immune Deficiency (PID)

Have a Question? Use Our New Chat Feature! Chat with our Medical Information Specialists by clicking on the Chat with us now available in the lower right corner of this screen.

What is PID Body

PID is a class of inherited disorders of the immune system that predispose individuals to an increased rate and severity of infection, immune dysregulation with autoimmune disease and malignancy. A high rate of comorbidities can occur with PID, including pulmonary diseases, rheumatological diseases, and autoimmune disease.

Infections occurs in various part of the body, usually the skin, sinuses, throat, ears, lungs, brain or spinal cord, urinary or intestinal tracts

These infections are characteristically repetitious, difficult to treat or unusually severe.

There are more than 450 primary immunodeficiencies recognized by the International Union of Immunological Societies.  There are approximately 500,000 people affected in the United States as estimated by NIH.

What are the symptoms of PID?

What is PID Body

  • Typical infections include those of the sinuses, ears, and lungs
  • Infections can lead to lung damage and shorten lifespan

PID, particularly CVID, is also associated with

  • Immune dysregulation and autoimmune disease
  • Autoimmune complications contribute to morbidity/mortality
  • Autoimmune hematologic complications often present as Immune Thrombocytopenia Purpura (ITP) and Autoimmune Hemolytic Anemia (AIHA)
  • Malignancy

PID is underdiagnosed and often has a long interval to diagnosis

  • PID; frequently go undetected in the primary care setting as the presentations may be perceived as routine infections
  • The time to diagnosis following symptom onset is on average 12.4 years
Physicians should consider evaluating for PID in patients who exhibit two or more of these warning signs.

Pediatric Patients

  1. Four or more new ear infections within 1 year
  2. Two or more serious sinus infections within 1 year
  3. Two or more months on antibiotics with little effect
  4. Two or more episodes of pneumonia within 1 year
  5. Failure of an infant to gain weight or grow normally
  6. Recurrent deep skin or recurrent organ abscesses
  7. Persistent thrush in mouth or fungal infection on skin
  8. Need for IV antibiotics to clear infections
  9. Two or more deep-seated infections including septicemia
  10. Family history of PID

Adult Patients

  1. Two or more new ear infections within 1 year
  2. Two or more serious sinus infections within 1 year, in the absence of allergy
  3. One episode of pneumonia per year for more than 1 year
  4. Chronic diarrhea with weight loss
  5. Recurrent viral infections (colds, herpes, warts, condyloma)
  6. Recurrent need for IV antibiotics to clear infections
  7. Recurrent deep skin or recurrent organ abscesses
  8. Persistent thrush or fungal infection of the skin or anywhere
  9. Infection with normally harmless tuberculosis-like bacteria
  10. Family history of PID
How is PID Treated?

Primary Therapy - Immunoglobulin

  • Ig therapy (IV or SC) is required for patients with PID characterized by impaired antibody production, usually accompanied by recurrent or unusually severe infection
  • Mandatory for patients with severe PIDs that directly impact B-cell function and antibody production
  • Increasingly seen as important in other PIDs in which antibody or B-cell dysfunction is implicated but may not be as apparent in conventional testing

Secondary/Supportive Measures

  • Antibiotics
  • Corticosteroids
  • Bronchodilators/Steroidal inhalants
  • Antivirals

Patients receiving Ig therapy should have regular monitoring of IgG trough levels, blood cell counts, and serum chemistry

Principle Principle Rationale
indications icon Indications
IVIg therapy is indicated as replacement therapy for patients with PID characterized by absent or deficient antibody production. This is an FDA-approved indication for IVIg, for which all currently available products are licensed. 
diagnosis icon Diagnosis
There are a large number of PID diagnoses for which IVIg is indicated and recommended. Many have low total levels of IgG, but some have a normal level with documented specific antibody deficiency.;

frequency icon Frequency of  IVIg treatment

IVIg is indicated as continuous replacement therapy for primary immunodeficiency. Treatment should not be interrupted once a definitive diagnosis has been established. 
Dose Icon Dose IVIg is indicated for patients with primary immunodeficiency at a starting dose of 400 to 600 mg/kg every 3 to 4 weeks. Less frequent treatment, or use of lower doses, is not substantiated by clinical data.
trough levels icon IgG trough levels IgG trough levels can be useful in some diagnoses to guide care but are NOT useful in many and should NOT be a consideration in access to IVIg therapy
site of care iconSite of Care The decision to infuse IVIg in a hospital, hospital outpatient, community office, or home based setting must be based upon clinical characteristics of the patient. 
route iconRoute Route of immunoglobulin administration must be based upon patient characteristics. The majority of patients are appropriate for intravenous and a subset are appropriate for subcutaneous therapy.
product icon Product IVIg is not a generic drug and IVIg products are not interchangeable. A specific IVIg product needs to be matched to patient characteristics to protect patient safety. A change of IVIg product should occur only with the active participation of the prescribing physician.

Table adapted from the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma & Immunology

For U.S. Healthcare Professionals only
For U.S. Healthcare Professionals only

The purpose of this CSL Behring Medical Affairs website is to support Healthcare Professionals with scientific information. This website is also a channel for U.S. Healthcare Professionals to submit questions or connect with CSL Behring U.S. Healthcare Professionals. The information provided is for educational purposes only and is not intended to promote any products. By continuing to use this site you are acknowledging that you are a US Healthcare Professional

toggle chat overlay
toggle chat overlay