Kikuchi disease

Kikuchi disease is a benign disease of the lymph nodes. The main sign of Kikuchi disease is usually swollen lymph nodes in the neck, which tend to develop suddenly. Other signs and symptoms may include:^[1][2]

• Fever
• Headache
• Nausea and vomiting
• Fatigue
• Joint pain
• Night sweats
• Rash
• Abdominal pain

The cause of Kikuchi disease is not known. However, scientists have proposed two possible explanations for the disease. Some believe that it may be due to a virus or other infectious agent. Features that support this theory include Kikuchi disease's frequent association with symptoms similar to those of certain viral infections and the condition's spontaneous resolution (goes away on its own) with or without specific treatment.^[1][2]

Others believe that it may be an autoimmune condition. According to this theory, Kikuchi disease occurs when one or more agents (for example, a virus) trigger a temporary immune response.^[1][2]

Kikuchi disease is not thought to be inherited. Most cases occur sporadically in people with no family history of the condition.^[2] Recently, a case study reporting two familial cases of Kikuchi disease was published in the literature, raising the hypothesis of genetic factors or perhaps a predisposing genetic background.^[3]

A diagnosis of Kikuchi disease is often suspected based on the presence of certain signs and symptoms. The only test available to confirm the diagnosis is a lymph node biopsy. However, other types of laboratory tests and imaging studies may be recommended to support the diagnosis and/or rule out other conditions that cause similar features.^[1][2]

Medscape Reference, a resource for medical professionals, offers more specific information about the diagnosis of Kikuchi disease. You may be asked to register to view the information but registration is free.

Kikuchi disease generally goes away (resolves) on its own within one to four months, with or without intervention. However, treatments are available to relieve some of the associated signs and symptoms. For example, nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to ease lymph node tenderness and fever. The use of corticosteroids has been recommended in severe cases.^[1][2] Hydroxychloroquine (an immunosuppressant), alone or in combination with other treatments, has also been successfully used.^[4]

Since there have been a few case reports of systemic lupus erythematosus (SLE or lupus) developing several years after recovery from Kikuchi disease, several medical researchers recommend long term follow-up to check for signs and symptoms of SLE.^[2][5]

Medscape Reference, a resource for medical professionals, offers more specific information regarding the treatment and management of Kikuchi disease. You may be asked to register to view the information, but registration is free.

The long-term outlook (prognosis) of Kikuchi disease is generally good. Lymph nodes usually return to normal size within 1-6 months after onset, although it may take longer. About 3% to 4% of people may develop Kikuchi disease again.^[1][2]

Kikuchi disease has been found to be associated with several autoimmune diseases, especially systemic lupus erythematous (SLE or lupus). Cases include those in which SLE began before Kikuchi disease, at the same time as Kikuchi disease, and in a few cases, several years after Kikuchi disease. Therefore some medical researchers recommend long term follow up to make check for the SLE.^[1][5] 

SLE is a chronic autoimmune disorder that may affect the skin, joints, kidneys, and other organs. Symptoms vary from person to person although almost all people with SLE have joint pain and swelling. Some develop arthritis. Other symptoms may include chest pain when taking a deep breath; general discomfort or ill feeling; and/or mouth sores.^[6]