Whipple disease

Whipple's disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. Caused by a bacterium called Tropheryma whipplei, the disorder can affect any system in the body, including the brain, eyes, heart, joints, and lungs, but usually occurs in the gastrointestinal system.

Neurological symptoms may include:

• Dementia
• Abnormalities of eye and facial muscle movements
• Headaches
• Seizures
• Loss of muscle control
• Memory loss
• Weakness
• Vision problems

Gastrointestinal symptoms may include:

• Diarrhea
• Weight loss
• Fatigue
• Weakness
• Abdominal bleeding and pain

Fever, cough, anemia, heart and lung damage, darkening of the skin, and joint soreness may also be present. Rarely, neurological symptoms may appear without gastrointestinal symptoms and can mimic symptoms of almost any neurological disease.

The disease is more common in men and neurological symptoms are more common in individuals who have severe abdominal disease.

The standard treatment is a prolonged course of antibiotics (up to two years), including penicillin and cefriaxone or doxycycline with hydroxychloroquine. Sulfa drugs (sulfonamides) such as sulfadizine or solfamethoxazole can treat neurological symptoms. Relapsing neurologic Whipple's disease (marked by bouts of worsening of symptoms) is sometimes treated with a combination of antibiotics and weekly injections of interferon gamma, a substance made by the body that activates the immune system.

Generally, long-term antibiotic treatment to destroy the bacteria can relieve symptoms and cure the disease. If left untreated, the disease is progressive and fatal. Individuals with involvement of the central nervous system generally have a worse outlook and may be left with permanent disability. Deficits may persist and relapses may still occur in individuals who receive appropriate treatment in a timely fashion. Prognosis may improve with earlier recognition, diagnosis, and treatment of the disorder.

How can I or my loved one help improve care for people with Whipple's disease?

Consider participating in a clinical trial so clinicians and scientists can learn more about Whipple's disease and related disorders. Clinical research uses human volunteers to help researchers learn more about a disorder and perhaps find better ways to safely detect, treat, or prevent disease.

All types of volunteers are needed—those who are healthy or may have an illness or disease—of all different ages, sexes, races, and ethnicities to ensure that study results apply to as many people as possible, and that treatments will be safe and effective for everyone who will use them.

For information about participating in clinical research visit NIH Clinical Research Trials and You. Learn about clinical trials currently looking for people with Whipple's disease at Clinicaltrials.gov.

Where can I find more information about Whipple's disease?

Information may be available from the following resources:

Genetic and Rare Diseases (GARD) Information Center

MedlinePlus

Content source: https://www.ninds.nih.gov/health-information/disorders/whipples-disease Accessed June 29, 2023.

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