What Is iMCD
Understanding iMCD and Why It Can Be a Long Road to Diagnosis
If you or a loved one has been diagnosed with idiopathic multicentric Castleman disease (iMCD), you probably have a lot of questions. Let’s start by helping you understand what iMCD is and how it differs from the infectious, autoimmune, cancerous, or other lymphoproliferative disorders it mimics.
iMCD can be very difficult to diagnose because it imitates the disorders mentioned above. The doctors will have to rule out all of these conditions before they can confirm iMCD, which can take a few days to several weeks.
Castleman Disease and Its Subtypes
Castleman disease (CD) is a rare condition that affects the lymph nodes.
Lymph nodes normally create immune cells that help to fight infections. In CD, there is an abnormal overgrowth of these cells, which causes the lymph nodes to appear swollen, resembling a tumor. However, these growths are not cancerous.
There are 2 main types of CD:
Unicentric Castleman disease (UCD)
UCD involves a single group of lymph nodes anywhere in the body that can be treated with surgical removal.
Multicentric Castleman disease (MCD)
MCD affects multiple groups of lymph nodes throughout the body. This condition is further categorized into 2 different types:
- Human herpesvirus‑8 (HHV-8)–positive MCD, which can cause Kaposi’s sarcoma—a rare cancer in which lesions grow on the skin, mouth, nose, throat, and other parts of the body
- Human immunodeficiency virus (HIV)–negative and HHV-8–negative MCD, also known as idiopathic multicentric Castleman disease (iMCD)
Idiopathic multicentric Castleman disease (iMCD)
iMCD is rare and can occur at any age, resulting from a cytokine storm that is often driven by interleukin‑6 (IL‑6).
Did you know?
There are approximately 30,000 Americans afflicted with one of the types of Castleman disease, which is roughly the same number as amyotrophic lateral sclerosis (ALS) and cystic fibrosis. Every year, approximately 1650 patients in the United States are diagnosed with new cases of MCD.
Understanding iMCD and the Importance of Cytokines
Idiopathic multicentric Castleman disease (iMCD) is a rare disorder in which the body overproduces various cytokines. Cytokines are powerful molecules that are normally secreted by the cells of the immune system in response to infectious or noninfectious agents.
If these overabundant cytokines are quickly released into the bloodstream, a cytokine storm can occur, with potentially severe or life-threatening effects. In the case of people with iMCD, the cytokine interleukin-6, or IL-6, may be responsible for the storm.
Symptoms of iMCD
Common Clinical Symptoms of iMCD
iMCD can be very difficult to diagnose because its presentation can vary, causing it to be mistaken for other conditions. If iMCD is not diagnosed and treated with the proper medication, it could lead to organ failure and other life-threatening conditions.
The following are symptoms commonly observed in patients with iMCD:
If you are a patient or a caregiver, be sure to report even common flu-like symptoms to your doctor.
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Diagnosis
An Excisional Biopsy Is the Most Effective Way to Confirm iMCD
A diagnosis of iMCD requires the examination of an affected lymph node. Your doctor may perform a needle biopsy (core biopsy) first to look for more common causes of your symptoms, but this is generally not sufficient to confirm iMCD.
During an excisional biopsy, a doctor will remove the affected lymph node. It is an invasive procedure that is performed under general anesthesia and may require hospitalization. Talk to your doctor if you have any questions about this procedure.
Important Safety Information
What is SYLVANT?
SYLVANT® (siltuximab) is a prescription medicine used to treat people with multicentric Castleman’s disease (MCD) who do not have human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8) infection. It is not known if SYLVANT is safe and effective in children.
Who should not take SYLVANT?
Do not take SYLVANT if you have had a severe allergic reaction to siltuximab or any of the ingredients in SYLVANT. See the Medication Guide for a complete list of ingredients in SYLVANT.
What are the possible side effects of SYLVANT?
SYLVANT may cause serious side effects, including:
Infections. SYLVANT may lower your ability to fight infections. Tell your healthcare provider right away if you have any signs or symptoms of an infection during treatment with SYLVANT.
Infusion and allergic reactions. If you have an infusion or allergic reaction while receiving SYLVANT, your healthcare provider will stop your infusion and treat your reaction. If you have a severe infusion or allergic reaction, your healthcare provider may stop your treatment completely. Tell your healthcare provider or get medical help right away if you have any of these symptoms during or after your infusion of SYLVANT:
- back pain
- chest pain or tightness
- nausea and vomiting
- flushing
- redness
- irregular heart beat (palpitations)
- trouble breathing
- wheezing
- dizziness or light-headedness
- swelling of the lips
- skin rash
- headache
- itching
The most common side effects of SYLVANT include:
rash, itching, upper respiratory tract infection, swelling, weight gain, and increased blood level of uric acid.
These are not all the possible side effects of SYLVANT. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
Before you take SYLVANT, tell your healthcare provider about all of your medical conditions, including if you:
- have an infection. You should not take SYLVANT if you have a severe infection.
- have had a recent vaccination or are scheduled to receive any vaccinations. You should not receive a live vaccine during your treatment with SYLVANT.
- have or have had any stomach or bowel (intestine) problems, such as diverticulitis or ulcers. Tell your healthcare provider if you have any pain or discomfort in your stomach.
- are pregnant or plan to become pregnant. It is not known if SYLVANT will harm your unborn baby. You should not become pregnant while receiving treatment with SYLVANT. Females who are able to become pregnant should use effective birth control during treatment with SYLVANT and for 3 months after stopping treatment.
- are breastfeeding or plan to breastfeed. It is not known if SYLVANT passes into your breast milk. You and your healthcare provider should decide if you will take SYLVANT or breastfeed. You should not do both.
Tell your healthcare provider about all the medicines you take,
including prescription and over-the-counter medicines, vitamins, and herbal supplements.
Please see full Prescribing Information for additional Important Safety Information.
Important Safety Information
What is SYLVANT?
SYLVANT® (siltuximab) is a prescription medicine used to treat people with multicentric Castleman’s disease (MCD) who do not have human immunodeficiency virus (HIV) and human herpesvirus-8 (HHV-8) infection. It is not known if SYLVANT is safe and effective in children.
Who should not take SYLVANT?
Do not take SYLVANT if you have had a severe allergic reaction to siltuximab or any of the ingredients in SYLVANT. See the Medication Guide for a complete list of ingredients in SYLVANT.
What are the possible side effects of SYLVANT?
SYLVANT may cause serious side effects, including:
Infections. SYLVANT may lower your ability to fight infections. Tell your healthcare provider right away if you have any signs or symptoms of an infection during treatment with SYLVANT.
Infusion and allergic reactions. If you have an infusion or allergic reaction while receiving SYLVANT, your healthcare provider will stop your infusion and treat your reaction. If you have a severe infusion or allergic reaction, your healthcare provider may stop your treatment completely. Tell your healthcare provider or get medical help right away if you have any of these symptoms during or after your infusion of SYLVANT:
- back pain
- chest pain or tightness
- nausea and vomiting
- flushing
- redness
- irregular heart beat (palpitations)
- trouble breathing
- wheezing
- dizziness or light-headedness
- swelling of the lips
- skin rash
- headache
- itching
The most common side effects of SYLVANT include:
rash, itching, upper respiratory tract infection, swelling, weight gain, and increased blood level of uric acid.
These are not all the possible side effects of SYLVANT. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
Before you take SYLVANT, tell your healthcare provider about all of your medical conditions, including if you:
- have an infection. You should not take SYLVANT if you have a severe infection.
- have had a recent vaccination or are scheduled to receive any vaccinations. You should not receive a live vaccine during your treatment with SYLVANT.
- have or have had any stomach or bowel (intestine) problems, such as diverticulitis or ulcers. Tell your healthcare provider if you have any pain or discomfort in your stomach.
- are pregnant or plan to become pregnant. It is not known if SYLVANT will harm your unborn baby. You should not become pregnant while receiving treatment with SYLVANT. Females who are able to become pregnant should use effective birth control during treatment with SYLVANT and for 3 months after stopping treatment.
- are breastfeeding or plan to breastfeed. It is not known if SYLVANT passes into your breast milk. You and your healthcare provider should decide if you will take SYLVANT or breastfeed. You should not do both.
Tell your healthcare provider about all the medicines you take,
including prescription and over-the-counter medicines, vitamins, and herbal supplements.
Please see full Prescribing Information for additional Important Safety Information.
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Biopsy
The removal and examination of tissue, cells, or fluids from the body.
Cytokines
Powerful molecules that are normally secreted by the cells of the immune system in response to infectious or noninfectious agents.
Cytokine storm
An overreaction of the body’s immune system in which too many cytokines are released into the bloodstream very quickly, with potentially severe or life-threatening effects.
Idiopathic
The underlying cause or origin of a disease is unknown.
Lymph nodes
Lymph nodes make immune cells that help the body fight infection.
Interleukin-6 (IL-6)
A key signaling molecule that helps to regulate the body’s immune system.
Pathologist
A doctor who evaluates the changes caused by disease in tissues and body fluids and helps reach a diagnosis.
Intravenous (IV) infusion
Administration of a drug solution into a vein.