How Is iMCD Diagnosed

iMCD may be confused with symptoms of other diseases

iMCD shares symptoms with many other conditions, which makes it difficult to diagnose. Often, people with iMCD see different types of doctors on their journey to diagnosis.
Your healthcare team will evaluate you for a variety of other conditions before reaching a diagnosis. Below are some conditions that iMCD can look like.

Autoimmune

Rheumatoid arthritis
Juvenile idiopathic arthritis
Adult-onset Still disease
Lupus
IgG4-related disease
Hemophagocytic lymphohistiocytosis/macrophage activation syndrome

Infectious

Acute Epstein-Barr virus
Acute human immunodeﬁciency virus

Malignant

Hodgkin lymphoma
Non-Hodgkin lymphoma
Follicular dendritic cell sarcoma
Polyneuropathy, organomegaly, endocrinopathy, monoclonal paraprotein, skin changes (POEMS) syndrome

Other

Malignancy-associated hemophagocytic lymphohistiocytosis
Autoimmune lymphoproliferative syndrome
Viral hemophagocytic lymphohistiocytosis
Human herpesvirus-8 (Kaposi sarcoma–associated herpesvirus)
Multicentric Castleman disease

Tracking symptoms may help you reach an accurate diagnosis sooner

In people living with iMCD, a delayed diagnosis is common. That’s why it’s important to track symptoms and report them to your healthcare team.

If you think you or someone you care for potentially has iMCD, your journey may start at a primary care physician’s office. You might meet doctors who specialize in different types of conditions, and they may conduct a few tests to help you get a proper diagnosis.

Doctors

A primary care physician will conduct blood tests/imaging and refer you or your loved one to a specialist
An infectious disease expert will rule out infections
A rheumatologist will rule out autoimmune conditions
A nephrologist will check to ensure that kidneys are functioning properly
A hematologist-oncologist will rule out cancerous conditions
A surgeon will remove a swollen lymph node
A pathologist will examine a swollen lymph node

This list is not a complete representation of all the doctors you might meet during your journey to diagnosis.

Tests

Physical examination of swollen lymph nodes, enlarged organs, and fluid accumulation
Blood tests to look for signs of infection, autoimmune conditions, and kidney damage
An x-ray, a positron emission tomography (PET) scan, or a computed tomography (CT) scan will reveal the presence of multiple swollen lymph nodes and enlarged organs

Biopsies

Fine needle aspiration (FNA) or core biopsy may be used to examine a swollen lymph node, but the results may be inconclusive. An FNA biopsy is when a small sample of fluid or tissue is removed from the swollen lymph node
An excisional biopsy of a swollen lymph node is the only definitive way to diagnose iMCD. An excisional biopsy is when the entire lymph node is removed

Guidelines that can help diagnose iMCD

For years, little has been known about iMCD, which has made the diagnostic journey frustrating.

Shortening the time from when symptoms first start to diagnosis can help improve long-term outcomes. Below are some guidelines your doctors may refer to when diagnosing this mysterious condition.

Major Criteria

A swollen lymph node should be removed and examined under a microscope to see if it has features consistent with Castleman disease
Imaging tests like computed tomography (CT) scans or positron emission tomography (PET) scans will help confirm the presence of multiple swollen lymph nodes throughout the body

Minor Criteria

At least 2 of 11 minor criteria must be met, such as the presence of enlarged liver, enlarged spleen, or other symptoms like fever, fatigue, or fluid accumulation
1 or more criteria must be a laboratory abnormality, such as anemia or increased protein in the urine

Exclusion Criteria

Castleman-like changes and clinical abnormalities may be present in several cancerous, infectious, and autoimmune conditions and should be ruled out by the process of elimination
Human herpesvirus-8 (HHV-8) must also be excluded

View Full Guidelines

“It took about 2 months to get diagnosed. The hematologist took a lymph node out for testing, and it came back that it was Castleman disease.”

-Frank, living with iMCD since 2018

REMEMBER:
iMCD is treatable. Learn more about the therapy options.