Many common signs and symptoms of Whipple's disease involve your gastrointestinal system and include:

• Diarrhea
• Abdominal cramping and pain, which tends to worsen after meals in some cases
• Weight loss, associated with the malabsorption of nutrients

Other frequent signs and symptoms associated with Whipple's disease include:

• Inflamed joints, particularly your ankles, knees and wrists
• Fatigue
• Weakness
• Anemia

Less common signs and symptoms are:

• Low-grade fever
• Cough
• Enlarged lymph nodes
• Difficulty walking
• Skin darkening (hyperpigmentation) in areas exposed to the sun, and in scars
• Visual impairment, including lack of control of eye movements
• Discomfort while breathing, due to inflammation of the membranes lining your lungs (pleurisy)
• Heart murmurs
• Enlarged spleen
• Seizures
• Confusion
• Memory loss

Symptoms tend to develop slowly over a period of many years in most people with the disease.

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The cause of Whipple's disease is infection with the bacterium Tropheryma whipplei. This bacterium can initially affect the mucosal lining of your small intestine, forming small lesions within the intestinal wall. With time, the infection can spread to other parts of your body.

Not much is known about the bacterium. Although it seems readily present in the environment, scientists don't really know where it comes from or how it's transmitted to humans.  Not everyone who carries the bacterium develops the disease. Some researchers believe that people with the disease may have a genetic defect in their immune system response that makes them more susceptible to becoming ill when exposed to the bacterium.

Whipple's disease is extremely uncommon. Recent estimates are that it affects one person in a million each year.

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Because so little is known about the bacterium that causes Whipple's disease, risk factors for the disease haven't been clearly identified. Based on available reports, it appears more likely to affect men than women, with the greatest prevalence in the 30 to 60 age group. It also seems to occur most commonly in whites in North America and Europe.

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Because Whipple's disease is potentially life-threatening yet treatable, contact your doctor immediately if you experience unusual signs or symptoms, such as unexplained weight loss or joint pain. Your doctor can perform tests to determine the cause of your symptoms.

Even after the infection is diagnosed and you're receiving treatment, let your doctor know if your symptoms don't improve. Sometimes antibiotic therapy isn't effective because the bacteria are resistant to the particular drug you're taking. Even after successful therapy the disease can recur, so it's important to watch for the re-emergence of symptoms that could be associated with the condition.

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Whipple's disease is rare and many doctors may be unfamiliar with the disorder, so it's often diagnosed in its late stages. However, the earlier the diagnosis, the better because of the serious health risks associated with not treating the condition. If your doctor is uncertain about the diagnosis, he or she may refer you to a doctor who specializes in digestive diseases or another specialist based on the symptoms you are having.

The process of diagnosing Whipple's disease typically includes the following tests:

• Physical exam. Your doctor will likely begin with a complete physical exam, looking for signs and symptoms that suggest the presence of the condition — for example, abdominal tenderness and skin darkening, particularly on sun-exposed parts of your body.

• Biopsy. An important step in diagnosing Whipple's disease is taking a tissue sample (biopsy), most often obtained from the lining of the small intestine. To do this, your doctor typically performs an upper endoscopy. The procedure uses a thin, flexible scope that passes through your mouth, throat, esophagus and stomach to your small intestine. The scope allows your doctor to view your digestive passages and obtain biopsies.

  During the procedure, tissue samples are removed from several sites within your intestine. This tissue is microscopically examined for the presence of disease-causing bacteria and their lesions, and specifically for Tropheryma whipplei bacteria. If biopsies of the small intestine don't confirm the diagnosis, your doctor might biopsy an enlarged lymph node or perform other tests if you have neurological symptoms, such as seizures.

• Blood tests. Your doctor may also order blood tests, such as a complete blood count (CBC). Blood tests can detect certain conditions associated with Whipple's disease, particularly anemia, which is a decline in the number of red blood cells, and low concentrations of albumin, a protein in your blood.

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The lining of your small intestine has fine hair-like projections (villi) that help your body absorb nutrients. Whipple's disease damages the villi, impairing nutrient absorption. Nutritional deficiencies are common in people with Whipple's disease and can lead to fatigue, weakness, weight loss and joint pain.

Whipple's disease is a progressive and potentially fatal disease. Although the infection is rare, associated deaths continue to be reported, due in large part to late diagnoses and delayed treatment. Death often is caused by the spread of the infection to the central nervous system, which can cause irreversible damage.

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Treatment of Whipple's disease is with antibiotics, which can destroy the bacteria causing the infection. Treatment is long term, generally extending over the course of a year or two, in an effort to completely eradicate the bacteria.  But relief from symptoms typically comes much quicker, often within a few days. Most people with no brain or nervous system complications recover completely after a full course of antibiotics.

Treatment course
Your doctor may choose from a number of antibiotics in treating Whipple's disease, either alone or in combination. A typical course of therapy requires hospitalization, with 14 days of intravenous (IV) ceftriaxone, followed by oral doses of trimethoprim-sulfamethoxazole, or TMP-SMX, (Bactrim, Septra) for one to two years on an outpatient basis. A shorter duration of antibiotic treatment may lead to a relapse.

Another common treatment begins with 14 days of IV infusions of streptomycin and penicillin G, followed by oral TMP-SMX for one to two years.

For the most serious cases, fluids and electrolyte replacement via IV drip may be added during initial hospitalization. Electrolytes are minerals such as sodium, potassium and calcium that maintain the balance of fluids in your body. 

Because of the lengthy use of antibiotics, your doctor will need to monitor your condition for development of resistance to the drugs.

When choosing antibiotics, doctors often select those that not only eradicate infections of the intestinal tract, but also cross the blood-brain barrier — a layer of tissue around your brain — in order to eliminate bacteria that may have entered your brain and central nervous system.

Symptom relief
Relief of symptoms tends to be rapid with proper antibiotic treatment. If you're experiencing joint pain, you may notice improvement in only a few days. Your diarrhea may go away within a week, and weight gain may begin just as soon.

Most other symptoms should subside within one to three months. But even though symptoms improve quickly, further laboratory tests may reveal presence of the bacteria for up to two years after you begin taking antibiotics. Follow-up testing will help your doctor determine when you can stop taking antibiotics. Regular monitoring can also indicate development of resistance to a particular drug, often reflected in a lack of improvement of your symptoms.

To manage joint pain, your doctor may recommend a nonsteroidal anti-inflammatory medication, such as ibuprofen (Advil, Motrin, others), as well.

If you have severe neurological symptoms or a long-lasting high fever, your doctor may also prescribe corticosteroids, which may help ease symptoms and reduce inflammation.

Even after successful treatment, Whipple's disease can recur. Doctors usually advise regular checkups. If you've experienced a recurrence, you'll need to repeat antibiotic therapy.

Taking supplements
Because of the nutrient-absorption difficulties associated with Whipple's disease, your doctor may recommend taking vitamin and mineral supplements to ensure adequate nutrition. Your body may require additional vitamin D, folic acid, calcium, iron and magnesium.

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