Whipple Disease

1 What is Whipple Disease?

Whipple disease is the result of a rare bacterial infection that damages the lining of the small intestine and may involve other organs of the body.

This disease is caused by a bacterial infection. Typical symptoms include diarrhea, inflamed and painful joints, weight loss, and abdominal pain.

The diagnosis is based on the results of biopsies of the small intestine. If left untreated, the disease is progressive and fatal.

Antibiotics can eliminate the infection, but the disease can recur.

Whipple disease affects mainly white men aged 30 to 60. It is caused by an infection with the organism Tropheryma whippelii. The infection almost always affects the small intestine but can affect other organs, such as

  • the heart,
  • lungs,
  • brain,
  • joints,
  • eyes.

2 Symptoms

Signs and symptoms of Whipple disease can vary widely from person to person. The most common symptoms of Whipple disease are:

  • diarrhea
  • weight loss caused by malabsorption

A person may not have diarrhea. Instead, other signs and symptoms of Whipple disease may appear, such as:

  • abnormal yellow and white patches on the lining of the small intestine
  • joint pain, with or without inflammation, that may appear off and on for years before other symptoms
  • fatty or bloody stools
  • abdominal cramps or bloating felt between the chest and groin
  • enlarged lymph nodes—the small glands that make infection-fighting white blood cells
  • loss of appetite
  • fever
  • fatigue, or feeling tired
  • weakness
  • darkening of the skin

People with a more advanced stage of Whipple disease may have neurologic symptoms—those related to the central nervous system—such as:

  • vision problems.
  • Memory problems or personality changes.
  • Facial numbness.
  • Headaches.
  • Muscle weakness or twitching.
  • Difficulty walking.
  • Hearing loss or ringing in the ears.
  • Dementia—People with dementia may not be able to think well enough to do normal activities such as getting dressed or eating.

Less common symptoms of Whipple disease may include:

  • Chronic cough,
  • Chest pain,
  • Pericarditis—inflammation of the membrane surrounding the heart,
  • Heart failure—a long-lasting condition in which the heart cannot pump enough blood to meet the body’s needs. Heart failure does not mean the heart suddenly stops working.

3 Causes

Bacteria called Tropheryma whipplei (T. whipplei) causes Whipple disease. T. whipplei infection can cause internal sores, also called lesions, and thickening of tissues in the small intestine.

The villi take on an abnormal, club like appearance and the damaged intestinal lining does not properly absorb nutrients, causing diarrhea and malnutrition.

Diarrhea is frequent, loose, and watery bowel movements. Malnutrition is a condition that develops when the body does not get the right amount of vitamins, minerals, and other nutrients it needs to maintain healthy tissues and organ function.

Over time, the infection spreads to other parts of the person’s body and will damage other organs.

4 Making a Diagnosis

A health care provider may use several tests and exams to diagnose Whipple disease, including the following:

  • medical and family history
  • physical exam
  • blood tests
  • upper GI endoscopy and enteroscopy

A patient may be referred to a gastroenterologist—a doctor who specializes in digestive diseases.

A health care provider may first try to rule out more common conditions with similar symptoms, including:

  • Inflammatory rheumatic disease—characterized by inflammation and loss of function in one or more connecting or supporting structures of the body.
  • Celiac disease—a digestive disease that damages the small intestine and interferes with the absorption of nutrients from food. People who have celiac disease cannot tolerate gluten, a protein in wheat, rye, and barley
  • Neurologic diseases—disorders of the central nervous system.
  • Intra-abdominal lymphoma—abdominal cancer in part of the immune system called the lymphatic system.
  • Mycobacterium avium complex—an infection that affects people with AIDS.

Medical and Family History

Taking a family and medical history can help a health care provider diagnose Whipple disease.

Physical Exam

A physical exam may help diagnose Whipple disease. During a physical exam, a health care provider usually

  • examines a patient’s body
  • uses a stethoscope to listen to sounds related to the abdomen
  • taps on specific areas of the patient’s body checking for pain or tenderness

Blood Tests

A technician or nurse draws a blood sample during an office visit or at a commercial facility and sends the sample to a lab for analysis. The health care provider may use blood tests to check for:

  • Malabsorption: When the damaged villi do not absorb certain nutrients from food, the body has a shortage of protein, calories, and vitamins. Blood tests can show shortages of protein, calories, and vitamins in the body.
  • Abnormal levels of electrolytes: Electrolytes—chemicals in body fluids, including sodium, potassium, magnesium, and chloride—regulate a person’s nerve and muscle function. A patient who has malabsorption or a lot of diarrhea may lose fluids and electrolytes, causing an imbalance in the body.
  • Anemia: Anemia is a condition in which the body has fewer red blood cells than normal. A patient with Whipple disease does not absorb the proper nutrients to make enough red blood cells in the body, leading to anemia.
  • T. whipplei DNA: Although not yet approved, rapid polymerase chain reaction diagnostic tests have been developed to detect T. whipplei DNA and may be useful in diagnosis.

Upper Gastrointestinal Endoscopy and Enteroscopy

An upper GI endoscopy and enteroscopy are procedures that use an endoscope—a small, flexible tube with a light—to see the upper GI tract. A health care provider performs these tests at a hospital or an outpatient center. The health care provider carefully feeds the endoscope down the esophagus and into the stomach and duodenum.

Once the endoscope is in the duodenum, the health care provider will use smaller tools and a smaller scope to see more of the small intestine. These additional procedures may include"

  • Push enteroscopy: Which uses a long endoscope to examine the upper portion of the small intestine.
  • Double-balloon enteroscopy: Which uses balloons to help move the endoscope through the entire small intestine.
  • Capsule enteroscopy: During which the patient swallows a capsule containing a tiny camera. As the capsule passes through the GI tract, the camera will transmit images to a video monitor. Using this procedure, the health care provider can examine the entire digestive tract.

A small camera mounted on the endoscope transmits a video image to a monitor, allowing close examination of the intestinal lining. A health care provider may give a patient a liquid anesthetic to gargle or may spray anesthetic on the back of the patient’s throat. A health care provider will place an intravenous (IV) needle in a vein in the arm or hand to administer sedation. Sedatives help patients stay relaxed and comfortable. The test can show changes in the lining of the small intestine that can occur with Whipple disease.

The health care provider can use tiny tools passed through the endoscope to perform biopsies. A biopsy is a procedure that involves taking a piece of tissue for examination with a microscope.

A pathologist—a doctor who specializes in examining tissues to diagnose diseases—examines the tissue from the stomach lining in a lab. The pathologist applies a special stain to the tissue and examines it for T. whipplei-infected cells with a microscope. Once the pathologist completes the examination of the tissue, he or she sends a report to the gastroenterologist for review.

The gold standard for the diagnosis of Whipple’s Disease is made by a biopsy of the duodenum, during an upper gastrointestinal endoscopy. Examination of the duodenal biopsy would show PAS-positive, non-acid fast organisms, within the macrophages.

  • Molecular tests, such as polymerized chain reaction (PCR) can be performed on a variety of specimens, such as saliva, gastric fluid, and intestinal fluid. It is important to note that a positive PCR test, in the absence of signs and symptoms does not indicate Whipple’s Disease. This could be due to the fact that the bacteria causing Whipple’s Disease may be found in normal healthy individuals, without causing any infection. A negative PCR test would rule out the possibility of the disease.
  • Biopsy of the lymph nodes can also be performed, which may demonstrate the presence of the bacteria within the lymph nodes.

5 Treatment

The health care provider prescribes antibiotics to destroy the T. whipplei bacteria and treat Whipple disease. IV antibiotics used to treat Whipple disease may include:

  • ceftriaxone (Rocephin)
  • meropenem (Merrem I.V.)
  • penicillin G (Pfizerpen)
  • streptomycin (Streptomycin)

After a patient completes the IV antibiotics, the health care provider will prescribe long-term oral antibiotics. Patients receive long-term treatment—at least 1 to 2 years—to cure the infection anywhere in the body. Oral antibiotics may include:

  • trimethoprim/sulfamethoxazole (Septra, Bactrim)—a combination antibiotic
  • doxycycline (Vibramycin)

Patients should finish the prescribed course of antibiotics to ensure the medication destroyed all T. whipplei bacteria in the body. Patients who feel better may still have the bacteria in the small intestine or other areas of the body for 1 to 2 years. A health care provider will monitor the patient closely, repeat the blood tests, and repeat the upper GI endoscopy with biopsy during and after treatment to determine whether T. whipplei is still present.

People may relapse during or after treatment. A health care provider will prescribe additional or new antibiotics if a relapse occurs. Some people will relapse years after treatment, so it is important for patients to schedule routine follow-ups with the health care provider. Most patients have good outcomes with an early diagnosis and complete treatment.

Health care providers treat patients with neurologic symptoms at diagnosis or during relapse more aggressively. Treatment may include:

  • a combination of antibiotics
  • hydroxychloroquine (Plaquenil)—an antimalarial medication
  • weekly injections of interferon gamma—a substance made by the body that activates the immune system
  • corticosteroids—medications that decrease inflammation

6 Prevention

Current medical research has not established a way of preventing Whipple’s Disease.

Medical screening at regular intervals with scans and physical examinations, are advised for those who have already endured the bacterial infection.

Farmers and sewage plant workers should contact their primary care physician early, if they are experiencing any of the symptoms of Whipple’s Disease.

7 Alternative and Homeopathic Remedies

Several alternative and homeopathic remedies exist for whipple disease.

Iron Intake

Since Whipple’s disease inhibits your body’s ability to absorb nutrients properly, it is essential to supplement it with minerals, like iron. You will be unable to properly digest iron, resulting in anemia and a low red blood cell count, which means you

Therefore, adding foods like

  • red meat,
  • eggs,
  • green leafy vegetables,
  • dried fruit,
  • liver,
  • molluscs,
  • lentils,
  • beans,
  • rtichokes,
  • turkey.

Fruits and Vegetables

One of the best ways to maintain a healthy lifestyle while dealing with Whipple’s disease is to ensure that your body has enough vitamins and nutrients.

Fruits and vegetables are often the best source of compact nutrients, and they also provide fiber, which helps the digestive processes within the body. Since Whipple’s disease affects the gastrointestinal system to such a high degree, any possible boost to digestive function is a good thing.

You may not fully absorb the beneficial nutrients in fruits and vegetables, but whatever your body is able to absorb will be beneficial. Furthermore, a lack of fruits and vegetables results in scurvy, which is a common associated issue with Whipple’s disease.

High-Protein Diet

Since Whipple’s disease affects various parts of the body and a number of the body’s processes, it is important to replenish the reserves of material to create new cells, muscle mass, blood vessels, etc.

Proteins are the building blocks of our body, and are an essential part in healing, as well as growth. By increasing the amount of protein we consume while suffering from Whipple’s disease, it is possible to stave off some of the more damaging symptoms and side effects of malabsorption. Some of the best protein-rich foods to eat are

  • turkey,
  • tuna,
  • salmon,
  • cheese,
  • pork loin,
  • tofu,
  • beans,
  • eggs,
  • yogurt,
  • milk,
  • nuts,
  • seeds.

Avoid High-Fat Foods

An unhealthy diet of fatty, greasy foods can make the symptoms of Whipple’s disease even worse, because they contribute to a weaker cardiovascular system, slow down your metabolic processes, and can contribute to many other health concerns that will further weaken your overall system. Therefore, maintaining an all-around healthy diet is the best way to increase your quality of life if you are suffering from Whipple’s disease.

Calcium and Magnesium

Consuming foods that are rich in calcium and magnesium can help improve your gastrointestinal system, along with many other aspects of human health. These are also two of the most important minerals that aren’t able to be absorbed by the body when suffering from Whipple’s disease.

Without these two minerals, your bone mineral density will decline, and the weakness already associated with the disease will be exacerbated.

Calcium-rich foods include:

  • milk,
  • yogurt,
  • cheese,
  • nuts,
  • various vegetables like broccoli and kale.

Magnesium-rich foods include:

  • spinach,
  • mackerel,
  • soy beans,
  • rice,
  • avocados,
  • bananas.

Vitamin Supplements

Since the body doesn’t absorb essential vitamins when Whipple’s disease occurs, it is important to use vitamin supplements to keep your levels healthy and normal. These sorts of vitamins can be found in foods if you specifically pay attention to which vitamins you need, but it is also easy to get vitamin supplements in organic food stores.

8 Risks and Complications

People with Whipple disease may have complications caused by malnutrition, which is due to damaged villi in the small intestine. As a result of delayed diagnosis or treatment, people may experience the following complications in other areas of the body:

  • long-lasting nutritional deficiencies,
  • heart and heart valve damage,
  • brain damage.

A person with Whipple disease may experience a relapse—a return of symptoms. Relapse can happen years after treatment and requires repeat treatments.