Kikuchi Fujimoto disease (KFD) is a rare condition that affects a person’s lymph nodes. As it shares symptoms with other conditions, it can be difficult to diagnose.

KFD is also known by other names, such as histiocytic necrotizing lymphadenitis or Kikuchi disease. KFD mainly occurs in people who are Japanese or East Asian, but it can occur in people of all ethnic backgrounds. It is also more common in females than males.

Swollen lymph nodes can be a symptom of KFD. However, many other conditions can cause a person to have swollen lymph nodes. This means KFD may be difficult for a doctor to diagnose.

Read on to learn more about KFD, including its symptoms, causes, and treatments.

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KFD is a very rare disease that can cause a person to have swollen lymph nodes. A person’s lymphatic system is part of their immune system. It consists of lymph vessels and lymph nodes. Lymph vessels carry a fluid called lymph around a person’s body.

Lymph contains white blood cells, which help the body fight infections. Structures called lymph nodes help filter the lymph fluid, by removing foreign substances. This can include infections and cancer cells. Lymph nodes also contain immune cells that help fight infections.

KFD most commonly affects the lymph nodes:

  • in the back of the neck
  • above the collarbones
  • in the armpits

KFD is a benign condition. This means that is it noncancerous. Research from 2022 notes that the most people with KFD are under 30 years old.

What causes KFD is currently unknown. Researchers believe that it may occur due to an immune response to a virus or autoimmune trigger.

Research from 2021 notes that KFD diagnoses have been associated with the following conditions:

Additional research from 2022 found that KFD could be associated with human immunodeficiency virus (HIV), parvovirus B19, and Torque teno/torque teno-like minivirus (TTV/TTMV).

KFD can cause a person to experience swollen lymph nodes. This swelling is generally unilateral, which means it only affects one side of the body. Evidence notes that KFD affects more than one area of lymph nodes in 22% of people.

Fever is the most common symptom of KFD. Other symptoms of KFD can include:

  • rashes
  • joint pain
  • fatigue
  • sore throat
  • upper respiratory problems
  • swelling of the liver and spleen
  • weight loss
  • night sweats
  • cough
  • headache
  • low white blood cell count
  • nausea
  • vomiting
  • diarrhea

Temporary skin issues can also occur alongside KFD. Evidence states that up to 40% of people with KFD also have a skin issue, such as:

  • a measles-like rash
  • rash on the face
  • spots or lesions on the face
  • hard bumps on the back and arms
  • scaling of the skin
  • itching
  • hair loss
  • ulcers on the back of the throat

Symptoms of KFD typically develop over 2–3 weeks. These symptoms generally clear up over the next 4 months.

There are various health conditions that can have similar symptoms to KFD. These include:

It is important that a doctor correctly diagnoses a person so that they can receive the right treatment. Research from 2016 notes that 58% of people with KFD within a specific study were misdiagnosed as having TB.

When diagnosing KFD, a doctor may perform a physical exam. They can feel a person’s lymph nodes to see if they are swollen.

A doctor may also order an imaging test, such as an ultrasound or magnetic resonance imaging (MRI) to check a person for enlarged lymph nodes.

A doctor confirms a KFD diagnosis by taking a sample from a person’s lymph node. This is known as a biopsy. The doctor then sends the biopsy sample to a laboratory for testing. If a person has KFD, their sample will show signs of cell death. Additionally, there will be a lack of granulocytes, a type of white blood cell.

There is no specific KFD blood test. However, a doctor can take a blood sample from a person to test their erythrocyte sedimentation rate (ESR). ESR tests how long it takes for a person’s red blood cells to settle on the bottom of a test tube.

A person’s red blood cells should settle slowly on the bottom of a test tube. However, if inflammation is present, their red blood cells will stick together. These clumps of cells will sink more quickly to the bottom of the test tube.

Research from 2017 notes that increased ESR was the most common observation in the KFD diagnoses included in the study.

KFD is a self-limiting condition. This means that it usually goes away by itself without treatment.

A person can take pain medication, such as acetaminophen or nonsteroidal anti-inflammatory drugs (NSAIDs) to help manage KFD-related pain. A doctor may treat a person who has severe symptoms or a reoccurrence of KFD. The KFD reoccurrence rate in adults is 3–4%.

Treatment options may include:

  • oral corticosteroids, a form of anti-inflammatory medication
  • intravenous immunoglobulin (IVIg), which provides a person with antibodies via a vein
  • hydroxychloroquine, a medication used for inflammation

In general, KFD resolves by itself over a number of months. However, it can cause a person to develop certain complications.

For example, evidence notes that KFD can develop into SLE months or years after the initial diagnosis. A person who has had KFD should see their doctor regularly to check for any signs of SLE.

In rare instances, a person who has KFD may develop complications such as:

  • meningoencephalitis, a condition that causes inflammation of the brain and the tissues that cover it
  • cerebellar ataxia, a condition that can cause loss of coordination and difficulty performing daily tasks
  • encephalitis, which is inflammation of the brain with central nervous system (CNS) lesions
  • aseptic meningitis, a form of meningitis not caused by bacteria

KFD is a rare condition that can cause a person to develop swollen lymph nodes. It’s diagnosed more often in young adults who are Japanese or East Asian. However, it can affect people of any age or racial background.

Symptoms of KFD can be similar to those of other health conditions. This can make it difficult for a doctor to correctly diagnose the condition. A correct diagnosis is important for a person to receive the right treatment.

KFD symptoms generally develop over a few weeks. Symptoms typically clear up by themselves up to 4 months after they develop. A person can treat pain due to KFD with pain medication. If a person has a more severe case of KFD, their doctor can treat the condition with various other medications.

In general, KFD resolves without issue. However, it can lead to certain people developing SLE. In rare instances, it can also cause a person to develop serious neurological complications.