About Castleman Disease
Castleman disease (CD) is a group of rare conditions that affect the immune system and is characterized by swollen lymph nodes and a broad range of inflammatory signs and symptoms. People with CD can experience a range of symptoms, including fever, night sweats, unexplained weight loss, and swollen lymph nodes. CD is often mistaken for other cancerous, autoimmune, and infectious disorders, making it extremely difficult to receive an accurate diagnosis.
There are 2 main types of Castleman disease:
Unicentric Castleman
disease (UCD)
UCD involves a single group
of lymph nodes.
Multicentric Castleman disease (MCD)
MCD affects multiple groups of lymph nodes. Idiopathic (ID-ee-oh-PATH-ik) multicentric (MULtee-SEN-trik) Castleman disease (iMCD) is a subtype of MCD. The word “idiopathic” means that the cause or origin of the disease is unknown.
Understanding iMCD
The cause of iMCD is unknown, and there are no known risk factors. There is no evidence that exposure to any food, lifestyle, or environmental factors is associated with iMCD.
Some people with iMCD have interleukin-6 (in-ter-LOO-kin-six), or IL-6, levels that are elevated. This may explain some of the symptoms they experience, such as swollen lymph nodes, fever, and night sweats.
What is IL-6?
- IL-6 is a small type of protein known as a cytokine (SY-toe-kyne). Normal levels of IL-6 are produced when the immune system responds to infections or inflammation
- Although the exact cause of iMCD is unknown, it is believed that a possible cause is the production of too much IL-6 in the body
- Our body’s immune response to infections or tissue damage begins in the lymph nodes with the release of cytokines such as IL-6. Elevated cytokine levels in people with iMCD can cause swollen lymph nodes throughout the body
What is a cytokine storm?
- A cytokine is a small protein that is very important in controlling the growth and activity of other cells in the immune system
- A cytokine storm is a severe immune system reaction that includes an excess release of cytokines, including IL-6, into the blood too quickly. A cytokine storm can occur in iMCD
iMCD can affect a person regardless of their age, gender, or race. Every year, approximately 1000 to 1100 people in the United States are diagnosed.
REMEMBER:
Getting to an iMCD diagnosis may be difficult—symptoms of iMCD can range from mild to severe and look like other conditions. That’s why it’s important to know the symptoms and share them with your healthcare team to prevent a delay in diagnosis.
