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Whipple's disease

Medically reviewed by Drugs.com. Last updated on Nov 8, 2022.

Overview

Whipple disease is a rare bacterial infection that most often affects your joints and digestive system. Whipple disease interferes with normal digestion by impairing the breakdown of foods, and hampering your body's ability to absorb nutrients, such as fats and carbohydrates.

Whipple disease can also infect other organs, including your brain, heart and eyes.

Without proper treatment, Whipple disease can be serious or fatal. However, a course of antibiotics can treat Whipple disease.

Small intestine lining

The small intestine has a ridged lining covered with tiny fingerlike projections called villi.

Symptoms

Common signs and symptoms

Digestive signs and symptoms are common in Whipple disease and may include:

Other frequent signs and symptoms associated with Whipple disease include:

Less common signs and symptoms

In some cases, signs and symptoms of Whipple disease may include:

Brain and nervous system (neurological) signs and symptoms may include:

Symptoms tend to develop slowly over many years in most people with this disease. In some people, symptoms such as joint pain and weight loss develop years before the digestive symptoms that lead to diagnosis.

When to see a doctor

Whipple disease is potentially life-threatening yet usually treatable. Contact your doctor 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. The disease can recur, so it's important to watch for symptoms that reappear.

Causes

Whipple disease is caused by a type of bacterium called Tropheryma whipplei. The bacteria affect the mucosal lining of your small intestine first, forming small sores (lesions) within the wall of the intestine. The bacteria also damage the fine, hairlike projections (villi) that line the small intestine.

Not much is known about the bacteria. Although they seem readily present in the environment, scientists don't know where they come from or how they're spread to humans. Not everyone who carries the bacteria 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 likely to become sick when exposed to the bacteria.

Whipple disease is extremely uncommon, affecting fewer than 1 in 1 million people.

Risk factors

Because so little is known about the bacteria that cause Whipple disease, risk factors for the disease haven't been clearly identified. Based on available reports, it appears more likely to affect:

Complications

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

Whipple disease is a progressive and potentially fatal disease. Although the infection is rare, associated deaths continue to be reported. This is 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.

Diagnosis

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

Treatment

Treatment of Whipple disease is with antibiotics, either alone or in combination, which can destroy the bacteria causing the infection.

Treatment is long-term, generally lasting a year or two, with the aim of destroying the bacteria. But symptom relief generally comes much quicker, often within the first week or two. Most people with no brain or nervous system complications recover completely after a full course of antibiotics.

When choosing antibiotics, doctors often select those that wipe out infections in the small intestine and also cross a layer of tissue around your brain (the blood-brain barrier). This is done to eliminate bacteria that may have entered your brain and central nervous system.

Because of the lengthy use of antibiotics, your doctor will need to monitor your condition for development of resistance to the drugs. If you relapse during treatment, your doctor may change your antibiotics.

Treatment for standard cases

In most cases, Whipple disease therapy begins with two to four weeks of ceftriaxone or penicillin given through a vein in your arm. Following that initial therapy, you'll likely take an oral course of sulfamethoxazole-trimethoprim (Bactrim, Septra) for one to two years.

Possible side effects of ceftriaxone and sulfamethoxazole-trimethoprim include allergic reactions, mild diarrhea, or nausea and vomiting.

Other medications that have been suggested as an alternative in some cases include oral doxycycline (Vibramycin, Doryx, others) combined with the antimalarial drug hydroxychloroquine (Plaquenil), which you'll likely need to take for one to two years.

Possible side effects of doxycycline include loss of appetite, nausea, vomiting and sensitivity to sunlight. Hydroxychloroquine may cause loss of appetite, diarrhea, headache, stomach cramps and dizziness.

Symptom relief

Your symptoms should improve within one to two weeks of starting antibiotic treatment and go away entirely within about one month.

But even though symptoms improve quickly, further lab tests may reveal the presence of the bacteria for two or more years after you begin taking antibiotics. Follow-up testing will help your doctor determine when you can stop taking antibiotics. Regular monitoring can also detect development of resistance to a particular drug, often indicated by a lack of improvement in symptoms.

Even after successful treatment, Whipple 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 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.

Preparing for your appointment

If you have signs and symptoms common to Whipple disease, make an appointment with your doctor. Whipple disease is rare, and the signs and symptoms can indicate other, more common disorders, so it can be hard to diagnose. As a result, it's often diagnosed in its later stages. However, an early diagnosis reduces the risk of serious health effects 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 to another specialist depending on the symptoms you're having.

The following information will help you prepare for your visit, and understand what to expect from your doctor.

Information to gather in advance

For signs and symptoms common to Whipple disease, some basic questions to ask your doctor include:

Don't hesitate to ask any other questions you have.

What to expect from your doctor

A doctor who sees you for possible Whipple disease is likely to ask a number of questions, such as:

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