What is Whipple's disease?

Whipple's disease is a multi-system infectious bacterial disease that interferes with the body's ability to metabolize fats. Caused by the bacterium 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 occur in up to 40 percent of individuals and may include dementia, abnormalities of eye and facial muscle movements, headaches, seizures, loss of muscle control, memory loss, weakness, and vision problems. Gastrointestinal symptoms may include diarrhea, weight loss, fatigue, weakness, and abdominal bleeding and pain. Fever, cough, anemia, heart and lung damage, darkening of the skin, and joint soreness may also be present. The disease is more common in men and neurological symptoms are more common in individuals who have severe abdominal disease. Rarely, neurological symptoms may appear without gastrointestinal symptoms and can mimic symptoms of almost any neurologic disease.

Is there any treatment?

The standard treatment for Whipple's disease 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.

What is the prognosis?

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 prognosis and may be left with permanent neurologic 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.

Symptoms of Whipple disease can vary from person to person and may affect many systems of the body. Some of the most common symptoms, occurring in the majority of people with Whipple disease, include:

• Weight loss.
• Diarrhea.
• Joint pain.

Other signs and symptoms may include:

• Neurological symptoms such as eye and facial muscle abnormalities, confusion, seizures, ataxia, memory loss, and vision impairment.
• Fatigue.
• Weakness.
• Abdominal bleeding.
• Abdominal pain.
• Fever.
• Loss of appetite.
• Darkening of the skin