Immunology


What are immune deficiencies?

Nearly everyone has suffered from colds, the flu, or sinus and ear infections. Just as many have been affected by cuts, scrapes and abrasions that become infected. Even in the case of more severe infections – such as pneumonia – we expect the cough and congestion to ‘run its course’, aided by prescription antibiotics, over-the-counter symptom remedies, and our body’s own immune system.

Recovery times vary, but the human body can usually rid itself of the infection-causing germs, and work to defend against future “bugs” and viruses. There are, however, instances in which the body cannot recover, and some of these apply to individuals with an immunodeficiency disease

When part of the immune system is either absent or not functioning properly, it can result in an immune deficiency disease. These diseases can be acquired or inherited. The inherited/genetic immune diseases are called primary immune deficiency diseases (PIDD). There are currently more than 150 different primary immune deficiencies.

The immune system is composed of white blood cells. These cells are made in the bone marrow and travel through the bloodstream and lymph nodes. They protect and defend against attacks by "foreign" invaders such as germs, bacteria and fungi. In the most common PIDDs, different forms of these cells are missing. This creates a pattern of repeated infections, severe infections and/or infections that are unusually hard to cure. These infections may attack the skin, respiratory system, the ears, the brain or spinal cord, or in the urinary or gastrointestinal tracts.

In some instances, PIDD targets specific and/or multiple organs, glands, cells and tissues. For example, heart defects are present in some PIDDs. Other PIDDs alter facial features, some stunt normal growth and still others are connected to autoimmune disorders such as rheumatoid arthritis.


When should I be worried about an immune deficiency?

Most severe PIDD appear in infancy but milder forms may not present till later in life. Some clues can help you decide if you may have an immune deficiency. Do the same symptoms keep coming back or never seem to completely clear? Is the infection severe enough to require hospitalization or intravenous antibiotics? Is there a family history of early infant death or of susceptibility to infection?

Important signs that may indicate a PIDD include:
  • Recurrent, unusual or difficult to treat infections
  • Poor growth or loss of weight
  • Recurrent pneumonia, ear infections or sinusitis
  • Multiple courses of antibiotics or IV antibiotics necessary to clear infections
  • Recurrent deep abscesses of the organs or skin
  • A family history of PIDD
  • Swollen lymph glands or an enlarged spleen
  • Autoimmune disease
An allergist / immunologist or a clinical immunologist has specialized training and expertise to accurately diagnose and coordinate a treatment plan for immune deficiencies.