Whipple's Disease

Whipple’s disease is an uncommon condition which affects several organs and systems in the body. In most cases the bowel is affected but the joints, eyes, heart, brain and lungs can also be affected. The disease is named after an American pathologist, Dr George Hoyt Whipple, who first described the disease in 1907. Whipple’s disease affects the body’s aptitude to absorb nutrients, causing problems related to deficiencies of vitamins, minerals and nutrients; known as malabsorption.

Whipple’s disease is very rare and it is estimated that there is less than 1 case per 1,000,000 people every year. It can affect people of different ages but it is most commonly found in middle-aged people. The disease tends to be more common in males, females and also among Caucasian populations.

Whipple’s disease is sometimes known as intestinal lipodystrophy.

What causes Whipple’s disease?

Whipple’s disease is brought on by infection through a strain of bacteria known as Tropheryma whippelii. The disease most commonly affects the small intestine, but it can cause problems in other organs and body systems. There is some evidence to suggest that family history may increase the risk of Whipple’s disease.

Symptom of Whipple’s disease

Symptoms of Whipple’s disease usually develop gradually and tend to begin with joint pain. Most other symptoms are linked to infection in the stomach and intestines, but as other systems may be affected other symptoms can develop. Symptoms include:

• Weight loss.
• Loss of appetite.
• Fatigue.
• Diarrhoea.
• Fever.
• Abdominal pain.
• Changes in the colour of the skin.
• Confusion and memory loss.
• Flatulence.
• Bloating.
• Chronic cough.
• Problems affecting the eyes.
• Enlarged lymph nodes.
• Fatty stools.
• Gastrointestinal bleeding.

Complications of Whipple’s disease

If left untreated, Whipple’s disease can cause severe complications, including:

• Brain damage.
• Damage to the heart.
• Malnutrition.
• Severe weight loss and anorexia in extreme cases.

How is Whipple’s disease diagnosed?

Whipple’s disease can be difficult to diagnose because if affects several organs and systems and there is no specific test to confirm a diagnosis. As the disease is so rare other conditions that cause related symptoms may be investigated first, in order to rule them out. Tests that may be used in the diagnostic process for Whipple’s disease include:

• Blood tests.
• Biopsy samples from the small intestine and other affected organs.
• Endoscopy.
• Polymerase chain reaction (known as PCR), which is used to detect the presence of Tropheryma whippelii bacteria.

Treatment for Whipple’s disease

The main treatment for Whipple’s disease is antibiotics, which are prescribed to fight the Tropheryma whippelii bacteria infection. However, it may be necessary to try a few different drugs to find the best course of antibiotics. If symptoms reappear during treatment a different drug will usually be trialled. Antibiotics are taken for at least 12 months and it can take two years for the small intestine to recover fully.

Depending on the severity of the condition additional treatments, including electrolyte, fluid replacement therapy and nutritional supplements may also be required. Whipple’s disease causes malabsorption, which prevents the body from being able to absorb important nutrients, vitamins and minerals. If a patient has a deficiency they may be advised to take vitamin and mineral supplements, including iron, magnesium and calcium supplements and vitamins B12 and D.

After the course of antibiotics has finished a repeat PCR test will be carried out.

If you are being treated for Whipple’s disease and your symptoms get worse, or you experience new symptoms, you should contact your doctor as soon as possible.

What is the outlook for people with Whipple’s disease?

Whipple’s disease is often able to be cured and managed effectively if the condition is diagnosed and treated early.

The outlook is poor if the condition is left untreated and complications can be serious and potentially life-threatening; most cases left untreated prove to be fatal. Patients should be monitored closely as relapse occurs in around 30 percent of people. Once treatment has started gastrointestinal symptoms tend to improve quickly, but other symptoms can take a long time to ease.