Granulomatous (gran-u-lo-mat-us) disease is an unusual kind of tissue inflammation. Its hallmark is collections or aggregates of immune cells that clump together to form nodular structures. These collections of cells are known as granulomas. They can be of different sizes and affect different tissues.
Why do granulomas form?
It is thought they form because the immune cells are not regulated properly or because the immune cells are attempting to contain or ‘wall-off’ an infection that the body cannot effectively deal with.
What are the symptoms of granulomatous disease?
The presence of these granulomas is associated with non-specific symptoms such as fever, tiredness, discomfort or pain. Other symptoms can depend on which tissues or organs are affected by the granulomas. These can include swelling of glands under the skin in the neck, armpits or groin. In other cases, if the granulomas form in the lungs, this might cause breathlessness. In the liver, there might be jaundice or abnormalities in the results of blood tests which your doctor takes (‘liver function tests’). If the inflammation is present for a long period of time and left untreated it can cause irreversible damage, such as scarring of the affected tissue or organ.
Where does granulomatous disease occur?
Granulomatous inflammation in primary immunodeficiency (PID) disorders can occur in any tissue or organ but is most frequently seen in the lungs, liver, spleen, lymph nodes (glands) and skin.
Which PIDs are particularly associated with granulomatous disease?
Two types of PID that are most frequently associated with the development of granulomas are chronic granulomatous disease (CGD) and severe forms of common variable immune deficiency (CVID).
This page was reviewed by the Medical Advisory Panel, April 2018.