Kikuchi-Fujimoto disease

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16Specialists8Treatment centers

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UniteRare data is sourced from FDA.gov, ClinicalTrials.gov, Orphanet, OMIM, and NORD.
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Overview

Kikuchi-Fujimoto disease (KFD), also called Kikuchi disease or histiocytic necrotizing lymphadenitis, is a rare, self-limiting condition that mainly affects the lymph nodes — the small glands that are part of your immune system. It was first described in Japan in 1972. The disease causes the lymph nodes, especially those in the neck, to become swollen and tender. Doctors believe it happens when the immune system overreacts, possibly triggered by a viral infection or an autoimmune process, though the exact cause is still not fully understood. The most common symptoms are swollen and painful lymph nodes in the neck, fever, and fatigue. Some people also develop a skin rash, night sweats, or weight loss. The condition most often affects young adults, particularly women, and is more commonly reported in people of Asian descent. Because its symptoms look similar to lymphoma (a type of blood cancer) and other serious conditions, getting the right diagnosis can take time and usually requires a lymph node biopsy. The good news is that Kikuchi-Fujimoto disease almost always goes away on its own within one to four months. Treatment focuses on relieving symptoms, such as using pain relievers and anti-inflammatory medicines. In more severe cases, doctors may prescribe corticosteroids like prednisone. Serious complications are rare, but the disease can occasionally come back, and a small number of patients may later develop lupus (an autoimmune disease), so follow-up care is important.

Also known as:

Key symptoms:

Swollen, tender lymph nodes, most often in the neckFever, sometimes highFatigue and low energySkin rash (usually red or pink spots)Night sweatsUnintentional weight lossHeadacheSore throatJoint pain or achesNauseaEnlarged lymph nodes in the armpits or groin (less common)

Clinical phenotype terms (50)— hover any for plain English
Cervical lymphadenopathyHP:0025289Abnormal lymph node morphologyHP:0002733Low-grade feverHP:0011134ChillsHP:0025143Malar rashHP:0025300Palpebral edemaHP:0100540
Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Kikuchi-Fujimoto disease.

View clinical trials →

No actively recruiting trials found for Kikuchi-Fujimoto disease at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Kikuchi-Fujimoto disease community →

Specialists

16 foundView all specialists →
YW
Yujia Wang
ESCONDIDO, CA
Specialist
1 Kikuchi-Fujimoto disease publication
VS
Vikas Sharma
Specialist
1 Kikuchi-Fujimoto disease publication
NS
Neeraj Sharma
Specialist
1 Kikuchi-Fujimoto disease publication
RR
Ritu Rani
Specialist
1 Kikuchi-Fujimoto disease publication
CC
Caiming Chen
Specialist
1 Kikuchi-Fujimoto disease publication
JX
Jingzhi Xie
Specialist
1 Kikuchi-Fujimoto disease publication
YX
Yanfang Xu
Specialist
1 Kikuchi-Fujimoto disease publication
HG
Hussain A Al Ghadeer
Specialist
1 Kikuchi-Fujimoto disease publication
SA
Sajjad M AlKadhem
Specialist
1 Kikuchi-Fujimoto disease publication
MA
Mohammed S AlMajed
DETROIT, MI
Specialist
1 Kikuchi-Fujimoto disease publication
HA
Hassan M AlAmer
Specialist
1 Kikuchi-Fujimoto disease publication
JA
Jaber A AlHabeeb
Specialist
1 Kikuchi-Fujimoto disease publication
SA
Suad H Alomran
Specialist
1 Kikuchi-Fujimoto disease publication
AA
Abdullah S AlMajed
Specialist
1 Kikuchi-Fujimoto disease publication
AK
Arvind Kumar
Specialist
1 Kikuchi-Fujimoto disease publication
LW
LI-Chieh Wang
HOLLIS, NY
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Travel Grants

No travel grants are currently matched to Kikuchi-Fujimoto disease.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Kikuchi-Fujimoto disease

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Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.How certain are you that this is Kikuchi-Fujimoto disease, and has lymphoma been fully ruled out?,Do I need a lymph node biopsy, and what will that procedure involve?,What symptoms should prompt me to go to the emergency room or call you right away?,Is there a risk that I could develop lupus, and how will you monitor me for that?,How long do you expect my symptoms to last, and when can I return to work or school?,Is there any treatment that will help me feel better faster, or should I just wait for it to resolve?,How often should I have follow-up appointments after I recover?

Common questions about Kikuchi-Fujimoto disease

What is Kikuchi-Fujimoto disease?

Kikuchi-Fujimoto disease (KFD), also called Kikuchi disease or histiocytic necrotizing lymphadenitis, is a rare, self-limiting condition that mainly affects the lymph nodes — the small glands that are part of your immune system. It was first described in Japan in 1972. The disease causes the lymph nodes, especially those in the neck, to become swollen and tender. Doctors believe it happens when the immune system overreacts, possibly triggered by a viral infection or an autoimmune process, though the exact cause is still not fully understood. The most common symptoms are swollen and painful ly

How is Kikuchi-Fujimoto disease inherited?

Kikuchi-Fujimoto disease follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Kikuchi-Fujimoto disease typically begin?

Typical onset of Kikuchi-Fujimoto disease is adult. Age of onset can vary across affected individuals.

Which specialists treat Kikuchi-Fujimoto disease?

16 specialists and care centers treating Kikuchi-Fujimoto disease are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.