What is Kikuchi Disease?

Kikuchi disease or Kikuchi-Fujimoto disease (KFD) is a rare condition that tends to appear in the Asian population and most often occurs in people of Japanese descent. It has occasionally shown up in other ethnic groups, but its greatest tendency is to occur in those with Asian ancestry. It is usually a condition that resolves on its own, within a few months of initial expression, but its symptoms make it somewhat complex. In particular, KFD presents with very swollen lymph nodes at the neck and shoulders and these can be mistaken for extremely severe conditions like lupus or lymphoma. Since treatment is so very different, and KFD usually does resolve on its own, ruling out Kikuchi disease through biopsy of a swollen node is often advised, though less often performed in populations at very low risk.

In addition to swollen lymph nodes at the neck, which may grow several centimeters in size, some people with Kikuchi disease may have other symptoms. Many people do have a fever that might increase during nighttime hours. Others report additional problems like stomach upset (vomiting, nausea). This may lead to weight loss. Another symptom sometimes associated with KFD is very sore throat, but this isn’t always present.

When appropriate diagnosis is made, treatment can still be variable. Some doctors prescribe non-steroidal anti-inflammatory drugs (NSAIDs) like ibuprofen or higher strength prescription only medicines to treat the discomfort associated with swollen lymph nodes. Ironically, in high doses these meds may increase stomach upset. Other favored treatments include fever reducers and pain relievers like acetaminophen. Continuing investigation in this area has suggested different medications that might be used instead, include intravenous immunoglobulin, which could treat more resistant cases.

Even without drug intervention, the majority of people get better. Very rarely, Kikuchi disease can cause complications in the lungs or liver, and these require additional intervention so that they do not cause mortality. It’s best to see a doctor if this disease is suspected or any time lymph nodes in this area become very swollen.

There is much discussion about Kikuchi disease origins and present thinking in the medical field leans toward viewing this condition as a complication or result of other viruses. Several candidate viruses are suggested including Epstein-Barr or HIV. Exactly how these viruses create the disease is not always clearly understood. Some disease specialists view the illness as a short duration autoimmune condition from which the body recovers.

One of the recommendations for people with Kikuchi disease is that they have regular check ups for several years after recovery. The reason for this recommendation is contentious and a matter of argument. There may be a link between onset of KFD and later development of lupus. The link is by no means clear or proven in all cases, but common sense would suggest having doctor care from time to time in the years following KFD to get early lupus treatment if it does occur.