Kikuchi Disease

Kikuchi Disease – No ticklish sensation here

Some of us might have heard about this, but most of us might have not. 

Introducing the Kikuchi Disease – a rare disease that affects the lymph nodes.

First discovered in Japan by two Japanese scientists in 1972, the Kikuchi-Fujimoto disease is a common disease in Japan. It is also known as histiocytic necrotizing lymphadenitis, which is a disease characterised by widespread lymphadenopathy – where the lymph nodes are at an abnormal size or consistency. 

It is rarely fatal nor harmful, and it is commonly associated with autoimmune disease in children while manifesting together with fever or flu-like symptoms. 

Kikuchi’s disease is not uncommon in the Singaporean population, and is fairly common in young people, predominantly young women in their 20-30s, in Asia.

Establishing an early diagnosis is crucial since the clinical presentation can mimic tuberculous lymphadenitis or malignant lymphoma. The diagnostic method of choice is excision biopsy.

Causes of Kikuchi Disease

The exact cause of the Kikuchi Disease is unknown (idiopathic). Many researchers and an overall and general cause for the disease is suggested to be a virus. 

Other research proposed autoimmune, genetic and autoimmune etiologies, and a widely accepted theory is the triggering of a self-limited autoimmune condition caused by an external agent. Autoimmune disorders happen when the body’s natural defences against “foreign” or invading organisms begin to attack healthy tissue for unknown reasons.

However, no infectious agent has yet been identified and autoimmunity remains hypothetical.

Symptoms of Kikuchi Disease

Being of a benign nature, the main sign for the Kikuchi disease is mainly the swollen lymph nodes (lymphadenopathy) in the neck, which happens suddenly, accompanied with the classic fever.

The fever is a primary symptom in most patients, but it is typically low grade and persists for about one week, though rarely for up to one month. 

Other signs and symptoms that may occasionally include:

  • Abdominal pain
  • Anaemia 
  • Arthritis
  • Diarrhoea
  • Fatigue
  • Headache
  • Hepatomegaly (enlarged liver)
  • Joint pain
  • Nausea and vomiting
  • Night sweats
  • Rash
  • Splenomegaly (enlarged spleen)
  • Weight loss

There is no clear way of knowing if you have the Kikuchi Disease, but through a CT scan, an MRI or an ultrasound can confirm the presence of enlarged lymph nodes – but it cannot confirm the diagnosis of the Kikuchi Disease.

This is because most of its symptoms are often mistaken for other conditions like tuberculous adenitis, lymphogranuloma venereum, Kawasaki disease, lymphoma or systemic lupus erythematosus (SLE). Fortunately, unlike most of the said conditions, Kikuchi Disease is not chronic and life-threatening, and it usually goes away on its own within 1 – 4 months. 

Affected patients should be followed for some years because they can develop systemic lupus erythematosus (SLE) and recurrences of Kikuchi’s disease can occasionally continue for many years. It may be rare, but it is discovered that the Kikuchi Disease can reoccur, and about 3% to 4% of people may develop Kikuchi Disease again.

Treatment for Kikuchi Disease

With or without intervention, Kikuchi Disease can resolve on its own but there is no effective treatment established. However, treatments are available to relieve some of the associated signs and symptoms. 

Treatment for Kikuchi Disease is symptomatic and supportive, and they include relieving any fever, flu symptoms and lymph node tenderness. For example, nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen can help to ease these symptoms.

Check up on your swollen lymph nodes

If you feel that you are down with a fever, with some tenderness in your lymph nodes, try to enquire with clinicians in WhatsDoc forums or consult an Ear Nose Throat Specialist on WhatsDoc to eliminate any other possibilities and get it treated immediately.

References:

https://rarediseases.info.nih.gov/diseases/6834/kikuchi-disease#:~:text=Kikuchi%20disease%20is%20a%20benign,%2C%20vomiting%2C%20and%20sore%20throat.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1481509/

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1849878/

https://emedicine.medscape.com/article/210752-overview

https://www.verywellhealth.com/kikuchi-disease-2860947

https://www.pathologyoutlines.com/topic/lymphnodeskikuchi.html

https://pubmed.ncbi.nlm.nih.gov/15858692/

https://pubmed.ncbi.nlm.nih.gov/16509908/

https://www.researchgate.net/publication/7878771_Kikuchi’s_disease_A_Singapore_case_series

http://www.smj.org.sg/sites/default/files/5101/5101cr7.pdf

https://www.medicinenet.com/kikuchi_disease/definition.htm

https://somepomed.org/articulos/contents/mobipreview.htm?35/63/36863?source=HISTORY