Whipple's Disease Causes, Signs, Symptoms, Diagnosis, Treatment, Prevention

Whipple's Disease Causes, Signs, Symptoms, Diagnosis, Treatment, Prevention

What Is Whipple’s Disease?

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

Whipple's Disease Causes, Signs, Symptoms, Diagnosis, Treatment, Prevention
Whipple's disease also can infect other organs, including your brain, heart, joints and eyes.

It is speculated that symptoms occur in people who have a genetic predisposition to developing the illness. White, middle-aged men (between 40 and 60) are more likely to contract the condition than any other group . In places that lack fresh water and proper sanitation, the rate for Whipple’s disease tends to be higher.

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

Read more: Whooping Cough (Pertussis) Vaccine, Causes, Symptoms, Diagnosis, Treatment, Prevention, Home Remedies

What causes Whipple's disease?

Bacteria called Tropheryma whipplei (T. whipplei) cause Whipple's disease. T. whipplei infection can cause internal sores, also called lesions, and the thickening of tissues. Villi, which are tiny fingerlike projections that line the small intestine, take on an abnormal, clublike appearance. The damaged intestinal lining fails to properly absorb nutrients, causing diarrhea and malnutrition.

Scientists are unsure how T. whipplei infects people. One theory is that some people are more vulnerable to Whipple's disease—probably due to genetic factors that influence the body's immune system. This theory is supported by the existence of a relatively high number of asymptomatic carriers—people who have the bacteria in their bodies but don't get sick. Also, the bacteria are more common in the environment—showing up in soil and sewage wastewater—than would be predicted based on the rareness of the disease. And while multiple cases of Whipple’s disease have occurred within the same family, no documentation exists of a person-to-person transmission.

Whipple's disease is extremely uncommon.

Whipple's Disease Risks

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
  • People ages 40 to 60
  • Whites in North America and Europe

What are the signs and symptoms of Whipple’s disease?

Classic signs and symptoms of Whipple’s disease include

  • periodic joint pain, with or without inflammation, that may persist for years before the appearance of other symptoms
  • chronic diarrhea, with or without blood
  • weight loss
  • abdominal pain and bloating
  • fever
  • fatigue
  • anemia—a condition in which the blood has a lower-than-normal number of red blood cells

Less common signs and symptoms of Whipple’s disease include

  • darkening of the skin
  • enlarged lymph nodes
  • chronic cough
  • chest pain
  • pericarditis—inflammation of the membrane surrounding the heart
  • heart failure

Neurologic symptoms of Whipple’s disease include

Neurologic symptoms occur in some people diagnosed with Whipple’s disease and can mimic symptoms of almost any other neurologic condition.

  • vision problems
  • dementia
  • facial numbness
  • headache
  • muscle weakness or twitching
  • difficulty walking
  • memory problems
Symptoms of neurologic, lung, or heart disease occasionally appear without gastrointestinal symptoms.

If the disease is not treated, this second stage may last for a number of years. Then more severe symptoms may occur due to the lack of nutrients reaching different parts of the body. Symptoms may occur in any part of the body, but most likely the heart, nervous system, brain, lungs, eyes, or skin. If the disease still is not treated, it usually causes death in this third stage.

When to see a doctor 

Whipple's 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 the re-emergence of symptoms.

How to Diagnose Whipple's Disease

The doctor will ask about your symptoms and medical history, and perform a physical exam. Tests may include:

  • Blood and stool tests-to look for problems with absorbing nutrients ( Malabsorption )
  • CT scan -a type of x-ray that uses a computer to make pictures of the inside of the body
  • Polymerase chain reaction (PCR) test-done on spinal fluid and the tissue of affected organs, to look for the bacteria that cause the disease
  • Biopsy -removal of a sample of tissue from your small intestine and other involved organs for testing

Treatments and drugs for Whipple’s Disease

Treatment of Whipple's 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, in an effort to destroy the bacteria.  But relief from symptoms typically 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 not only wipe out 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.

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's disease therapy begins with 14 days of intravenous (IV) ceftriaxone (Rocephin). Following that initial therapy, you'll likely take an oral course of sulfamethoxazole-trimethoprim, or SMX- TMP, (Bactrim, Septra), for one to two years. A shorter duration of antibiotic treatment may lead to a relapse.

Possible side effects of ceftriaxone and SMX- TMP include mild diarrhea, nausea and vomiting.

Treatment for severe cases 

If you have neurologic symptoms, you may be started immediately on a 12- to 18-month course of oral doxycycline (Vibramycin) combined with the antimalarial drug hydroxychloroquine (Plaquenil). You'll also be given long-term antibiotics that can enter the cerebrospinal fluid and brain, such as TMP-SMX.

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 laboratory tests may reveal 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 indicate development of resistance to a particular drug, often reflected in a lack of improvement of your symptoms.

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.

How to Prevent Whipple's Disease

Currently, there is no known way to prevent Whipple's disease.

Possible Complications

  • Brain damage
  • Heart valve damage (from endocarditis)
  • Nutritional deficiencies
  • Symptoms return (which may be because of drug resistance)
  • Weight loss

What is the likely outcome for people with Whipple's disease?

After treatment, the likely outcome for most people with Whipple's disease is good. Most symptoms disappear in about 1 month. Relapse is common, however, highlighting the need to closely watch for a return of symptoms. Endoscopy with small intestinal biopsy followed by PAS staining and electron microscopy or PCR testing should be repeated 1 year after the start of treatment.

People with neurologic Whipple's disease who relapse tend to have much poorer health outcomes, including serious neurologic symptoms and even death; therefore, some scientists argue that all cases of Whipple's disease should be considered neurologic. Relapsing neurologic Whipple’s disease is sometimes treated with a combination of antibiotics and weekly injections of interferon gamma (IFN╬│)—a substance made by the body that activates the immune system.

Long-Term Outlook of Whipple's Disease

After treatment begins, many of the symptoms will go away within a month. The best thing you can do is to faithfully take your antibiotics. Relapses are common, and when they occur, additional symptoms, such as neurological problems, can show up as well.