What Is a Primary Immunodeficiency Disease?

A primary immunodeficiency disease (PIDD) is a genetic problem that causes part of the immune system to malfunction. Alternatively, part of the immune system may be absent. Primary immunodeficiency diseases can range from mild to severe. Allergists typically diagnose severe PIDDs when the patient is quite young, but mild PIDDs may not be diagnosed until later in life as the health effects become more apparent.

Types of PIDDs

PIDDs can occur when there is a mutation in the genetic code. There are more than 300 types of PIDDs. Allergy specialists have categorized them into six main groups.

  • B cell (antibody) deficiencies
  • T cell deficiencies
  • B and T cell combination deficiencies
  • Complement deficiencies
  • Defective phagocytes
  • Idiopathic (unknown PIDD)

B and T cells, also called lymphocytes, are adaptive immune cells that fight off invading organisms in the body. Phagocytes are another specialized cell of the immune system. It engulfs and absorbs small cells like bacteria.

Signs of PIDDs

The defining characteristic of primary immunodeficiency diseases is having a higher susceptibility to infections. People with PIDDs may get infections more frequently, have a harder time fighting them off, and suffer from them for a longer period of time. Some people with PIDDs experience the following health issues:

  • Eight or more new ear infections within one year.
  • Two or more serious sinus infections within one year
  • Two or more months of antibiotics resulting in marginal improvement
  • Two or more bouts of pneumonia within one year
  • Recurrent, deep skin or organ abscesses
  • Failure of an infant to gain weight or grow normally
  • Persistent thrush in mouth or elsewhere on skin after age one

Additionally, patients with PIDDs are more susceptible to these infections:

  • Pneumonia
  • Bronchitis
  • Sinus infections
  • Skin infections
  • Meningitis
  • Ear infections

Treatments for PIDDs

Each treatment plan for a PIDD is personalized to meet the needs of the individual patient. The main goals for treatment include:

  • Improving the function of the immune system
  • Preventing and treating infections
  • Treating the underlying cause, if any

An allergist might recommend these therapies for PIDDs:

  • Immunoglobulin therapy: The introduction of antibody proteins through IV or subcutaneous infusion.
  • Interferon-gamma therapy: An injected therapy that stimulates the immune system. Appropriate for patients with chronic granulomatous disease.
  • Growth factors: A treatment that increases the amount of white blood cells.

If you or your child has been diagnosed with a primary immunodeficiency disease, you can find the supportive care you need at Allergy & Asthma Specialists SM. Our allergists are available in multiple locations in Pennsylvania, including Blue Bell, Center City, Lansdale, Philadelphia, Jenkintown, Pottstown, King of Prussia, and Collegeville, Pennsylvania. New and current patients can reach our office at 1(800)86-COUGH.

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