GARD Rare Disease Information - Chronic active Epstein-Barr virus infection - National Organization for Rare Disorders (2023)

Disease Overview

Chronic active Epstein-Barr virus infection(CAEBV) is a very rare complication of anEpstein Barr virus(EBV) infection. Symptoms of CAEBV may include fever, swollen lymph nodes, and an enlarged liver and/or spleen. More serious complications may include anemia, nerve damage, liver failure, and/or interstitial pneumonia. Symptoms may be constant or come and go, and tend to get worse over time. CAEBV occurs when the virus remains ‘active’ and the symptoms of an EBV infection do not go away. It is diagnosed based on the symptoms, clinical exam, and blood tests that show EBV DNA remaining at high levels for at least 3 months. Treatment is focused on managing the symptoms.The most well-documented, effective treatment for CAEBV is hematopoietic stem cell transplantation.[8879][15026][15027]

Some people with fatigue alone are mistakenly thought to have CAEBV. Very specific testing looking for the level of EBV DNA is necessary to diagnose CAEBV.[15027]


What is the life expectancy of someone with chronic Epstein-Barr virus? ›

Chronic active Epstein-Barr virus infection often results in poor prognosis. A large cohort study [18] in Japan reported that 43% of patients died during follow-up periods that ranged from 5 months to 12 years after the onset of severe CAEBV infection.

What is chronic active Epstein Barr virus disease? ›

Chronic active Epstein–Barr virus (CAEBV) disease is a rare disorder in which persons are unable to control infection with the virus. The disease is progressive with markedly elevated levels of EBV DNA in the blood and infiltration of organs by EBV-positive lymphocytes.

How rare is chronic active Epstein Barr? ›

Chronic active Epstein-Barr virus infection (CAEBV) is a very rare complication of an Epstein Barr virus (EBV). Most people (about 95% by adulthood) become infected by EBV at some point of their lives and never have any health problems.

How do you treat chronic active Epstein Barr virus? ›

At present, hematopoietic stem-cell transplantation is the only curative therapy, and it is critical to make a proper diagnosis and initiate transplantation before the disease progresses to an irreversible stage.

Is chronic Epstein-Barr a disability? ›

Epstein-Barr virus (EBV) can cause chronic fatigue, joint pain and difficulty concentrating, but because these symptoms and others rarely last even 12 months, it is unlikely that an individual would be considered disabled with a diagnosis of EBV alone.

Is chronic Epstein-Barr virus a disability? ›

If you suffer from chronic symptoms of EBV and have a medically determinable impairment, you may be eligible for long-term disability benefits. Long-term disability claims for EBV are often denied, but you should not give up on your case.

What are the 4 stages of Epstein-Barr virus? ›

For EBV we have a six-stage model of infection (naive Blast, GC, memory, Immediate early lytic, Early lytic and Late lytic) where each stage may or may not be regulated by the immune response.

What is the difference between Epstein Barr and chronic Epstein Barr? ›

In very rare cases, EBV infection can lead to a chronic condition called chronic active EBV (CAEBV). CAEBV is characterized by ongoing symptoms and blood test results that show an active EBV infection. CAEBV starts out as a typical EBV infection.

What kind of doctor treats chronic Epstein Barr virus? ›

For Epstein-Barr virus, you may be treated by a primary care provider (PCP), such as a family practitioner, an internist, or a child's pediatrician. If the symptoms of EBV become chronic, you may be referred to an infectious-disease specialist or an immunologist (also called an allergist/immunologist).

Can you live with chronic Epstein-Barr virus? ›

Chronic active Epstein-Barr virus infection (CAEBV) is a rare and lethal condition caused by persistent Epstein-Barr virus (EBV) infection. Signs and symptoms of CAEBV infection include fever, lymphadenopathy, and hepatosplenomegaly.

What does chronic Epstein-Barr feel like? ›

People with chronic active Epstein-Barr virus can have persistent or intermittent symptoms of infectious mononucleosis. They can have a recurrent fever, enlarged lymph nodes, enlarged spleen, and EBV hepatitis (liver inflammation).

What foods are not good for Epstein-Barr virus? ›

Avoiding sugar is also important for managing EBV symptoms, as sugar can increase inflammatory responses, impacting the immune system. Drinking water is also crucial in managing symptoms related to EBV.

What causes Epstein Barr to flare up? ›

EBV never truly goes away. Even if the symptoms subside, the virus will remain inactive inside your body until it is reactivated by a trigger. Some triggers include stress, a weakened immune system, taking immunosuppressants, or hormonal changes such as menopause.

What are the neurological symptoms of Epstein-Barr virus? ›

The clinical presentation is dominated by acute cerebellar ataxia with febrile illness, trunk ataxia, nystagmus, intentional tremor, headache, and altered mental status. The condition is more common in children and young adults.

Can Epstein Barr cause weight gain? ›

Persistent infections such as Lyme, Epstein Barr Virus etc. can be a source of inflammation and hence lead to weight gain.

What is the mortality rate of EBV? ›

It is now known to cause approximately 1.8% of all cancer deaths worldwide as well as being strongly associated with the development of multiple sclerosis. It is currently estimated that EBV-associated disease results in ~160,000 deaths per year.

Is chronic Epstein-Barr curable? ›

There is no cure for Epstein-Barr virus and there is no vaccine to prevent the spread of the virus. Treatment addresses the symptoms of the virus and symptoms should go away after two to four weeks.

Can you live a normal life with Epstein-Barr virus? ›

The prognosis for Epstein-Barr virus infection is good. Almost all people infected with EBV recover completely in about 1-3 months. Neurological changes usually completely resolve, although a few adults may have some deficits. Although most infections become latent, most remain asymptomatic.


Top Articles
Latest Posts
Article information

Author: Barbera Armstrong

Last Updated: 14/10/2023

Views: 5343

Rating: 4.9 / 5 (59 voted)

Reviews: 90% of readers found this page helpful

Author information

Name: Barbera Armstrong

Birthday: 1992-09-12

Address: Suite 993 99852 Daugherty Causeway, Ritchiehaven, VT 49630

Phone: +5026838435397

Job: National Engineer

Hobby: Listening to music, Board games, Photography, Ice skating, LARPing, Kite flying, Rugby

Introduction: My name is Barbera Armstrong, I am a lovely, delightful, cooperative, funny, enchanting, vivacious, tender person who loves writing and wants to share my knowledge and understanding with you.