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Kikuchi Disease - Causes, Symptoms, Diagnosis, and Management

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An unknown condition or infection of the lymph nodes is known as Kikuchi disease, which is benign (noncancerous). Read on to know more.

Medically reviewed by

Dr. Kaushal Bhavsar

Published At December 15, 2022
Reviewed AtDecember 15, 2022

What Is Kikuchi Disease?

Kikuchi's disease, which is also known as histiocytic necrotizing lymphadenopathy, and Kikuchi-Fujimoto disease. It is a rare and benign (noncancerous, nonmalignant) disorder affecting most young female adults. Research suggests that it may be caused by an infection or an autoimmune disorder, although the exact cause is unknown. Due to similar symptoms, cervical adenopathy is often misdiagnosed as malignant lymphoma. As a result of these lesions or tissue anomalies, the lymph nodes enlarge (lymphadenopathy), become inflamed and become painful. The precise cause of Kikuchi's illness is unknown. A misdiagnosis of a malignant lymphoma could be the most serious threat.

This condition was discovered in Japan in 1972 and has since spread to other parts of the world. The overall picture points to virus infection; autoimmune factors may also be involved. However, no infectious agent has been identified, and autoimmunity is still a theory. Experts generally agree that Kikuchi disease is caused by unidentified agents that trigger self-limited infections, chemicals, plastics, and neoplastic (abnormal tissue growth).

What Are the Causes of Kikuchi Disease?

The precise cause of Kikuchi's disease is unknown or idiopathic. Many researchers believe that this disorder is caused by a virus. Others, however, believe that the disorder is a self-limiting autoimmune condition similar to systemic lupus erythematosus. Autoimmune disorders develop when the body's natural defenses against "foreign" or invading organisms begin to attack healthy tissue for unknown reasons. It generally affects young women. Even though the etiology is unknown, it is proposed that it is a hyperimmune reaction to many physical, chemical, and neoplastic agents.

What Are the Signs and Symptoms of Kikuchi Disease?

The most common symptom is patients present with unilateral tender cervical lymphadenopathy. Lymph nodes are small oval structures found throughout the body that filter lymph fluid, fight infection, and produce white blood cells and blood plasma cells. In addition to abdominal swelling and pain, the lymph nodes near the salivary glands and on the side of the neck are frequently affected as well.

It does not seem to be contagious, but affected individuals may develop mild fevers, night sweats, muscle pains (myalgia), and rashes. Headaches, fatigue, joint pain (arthralgia), nausea, and vomiting are less common symptoms. Affected individuals may experience abnormal liver or spleen enlargement in some cases (hepatosplenomegaly). It can also cause anemia and neutropenia, and rarely patients may present with atypical lymphocytosis. Kikuchi's disease symptoms may appear gradually over two to three weeks.

For unknown reasons, Kikuchi's disease generally resolves spontaneously within a few weeks or months without further treatment (self-limited disease).

How to Diagnose Kikuchi Disease?

The diagnosis usually includes a thorough medical history and physical examination. Kikuchi disease is frequently misdiagnosed as lymphoma or systemic lupus erythematosus due to its symptoms and difficulty in diagnosis. The presence of enlarged lymph nodes can be confirmed with the following tests.

  • Ultrasound - It is imaging that creates pictures of the body's internal structures using sound waves.

  • CT (Computed Tomography) Scan - It is an effective diagnostic tool for locating illnesses and wounds. It is a painless, non-invasive procedure that produces images of a cross-section of the body using X-rays and computers.

  • MRI (Magnetic Resonance Imaging) - A three-dimensional, highly detailed anatomical image is produced using this non-invasive imaging technology.

The tests mentioned above could only determine the presence but not the diagnosis.

  • Lymph Node Biopsy - The only way to ensure that your symptoms are caused by Kikuchi disease is for the doctor to remove a lymph node and examine the tissues inside. That is lymph node biopsies. Lymph nodes are immune system components that aid the body in recognizing and combating infections. Lymph node biopsies may involve the doctor removing the entire lymph node or tissue samples from the swollen node. This procedure takes around ten to fifteen minutes. The physician will clean the biopsy site and numb the area while the patient is lying down. A fine needle will be inserted into their lymph node to extract a sample of cells. They will then remove the needle and apply a bandage to the surgical site. To determine whether or not a patient is infected, tissue is examined under a microscope.

Fortunately, unlike lymphoma and lupus, Kikuchi's disease is not fatal or chronic.

What Are the Risks of Kikuchi Disease?

Kikuchi disease has been reported in people of all races all over the world. Kikuchi disease was previously thought to affect more women than men. However, new evidence suggests that it does not affect one sex more than the other. There is no age limit for Kikuchi disease, but it most commonly affects young adults.

What Are the Treatments for Kikuchi Disease?

Kikuchi's disease is treated with symptomatic and supportive care. The disorder usually resolves on its own after a few weeks or months. Pain, tenderness, and fever caused by lymphadenopathy can be treated with Analgesics, Antipyretics, and nonsteroidal anti-inflammatory drugs (NSAIDs). A recurrence of Kikuchi's disease is extremely rare.

Kikuchi disease treatment consists of reducing fever, flu symptoms, and lymph node tenderness. Ibuprofen and other nonsteroidal anti-inflammatory drugs (NSAIDs) can help with these symptoms.

What Are the Prognosis and Management of Kikuchi Disease?

Usually, the prognosis is good in Kikuchi's disease, but few patients develop recurrent lymphadenopathy, which should be removed. It is rare for patients with immunodeficiency to have multiple organs involved with this disease, which can lead to a poor prognosis.

Currently, no specific management is available for Kikuchi disease due to its self-limited nature and unknown etiology.

Conclusion

This disease is also known as histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease. It is a benign disease with spontaneous remission, which is commonly seen in young women. It usually affects the unilateral post-cervical lymph node. This condition is a rare, idiopathic, self-limiting lymphadenitis. Kikuchi was first found in Japan. Kikuchi disease has no known cause, though infectious and autoimmune etiologies have been proposed. Kikuchi disease occurs concurrently with or after conditions that could have acted as triggers, such as COVID-19 infection and fever. The treatment of Kikuchi's disease consists of symptomatic and supportive measures, and the disorder usually resolves on its own.

Dr. Kaushal Bhavsar
Dr. Kaushal Bhavsar

Pulmonology (Asthma Doctors)

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