Whipple's disease

Description

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

Whipple's disease can also infect other organs, including the brain, heart, and eyes.

Without the proper treatment, Whipple's disease, which may be severe or fatal. However, a course of antibiotics can treat the disease Whipple.

Symptoms

Common signs and symptoms

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

  • Diarrhea
  • Stomach cramping and pain, which may be worse after meals
  • Weight loss, associated with poor absorption of nutrients

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

  • Inflammation of the joints, especially the ankles, knees and wrists
  • Fatigue
  • Weakness
  • Anemia

Less common signs and symptoms

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

  • Fever
  • Cough
  • Enlargement of the lymph nodes
  • Darkening of the skin in sun-exposed areas and in the scars
  • Pain in the chest

The brain and nervous system (neurological) symptoms and signs may include:

  • Difficulty walking
  • Vision problems, including the lack of control of eye movements
  • The confusion
  • Memory loss

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

When to see a doctor

Whipple's disease is life-threatening, but they can usually be treated. Contact your doctor if you experience any unusual signs or symptoms, such as unexplained weight loss or pain in the joints. Your doctor can perform tests to determine the cause of your symptoms.

Even after the infection is diagnosed and you are receiving treatment, tell your doctor if your symptoms do not improve. Sometimes the therapy with antibiotics is not effective because the bacteria are resistant to the particular drug you are taking. The disease can recur, so it is important to be aware of the symptoms reappear.

Causes

Whipple's disease is caused by a type of bacteria called Tropheryma whipplei. The bacterium affects the lining of the mucosa of the small intestine in the first place, the formation of small ulcers (lesions) in 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. Though they seem to be easily present in the environment, scientists do not know where they come from or how it spread to humans. Not all those who carry the bacteria develops the disease. Some researchers believe that people with the disease may have a genetic defect in the response of your immune system, which makes them more likely to get sick when exposed to the bacteria.

Whipple's disease is extremely rare, affecting less than 1 in 1 million people.

Risk factors

Because so little is known about the bacterium that causes Whipple's disease, risk factors for the disease have not been clearly identified. Based on the available reports, it seems more likely to be affected:

  • Men between ages 40 to 60
  • The white people in North America and Europe
  • Farmers and other people who work outdoors, and have frequent contact with the wastewater and sewage

Complications

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

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

Whipple's disease

Diagnosis

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

  • Physical exam. Your doctor usually starts with a physical examination. He or she will look for signs and symptoms that suggest the presence of this condition. For example, the doctor may look for the stomach tenderness, and the darkening of the skin, especially in sun-exposed parts of your body.
  • The biopsy.An important step in the diagnosis of Whipple's disease is to take a sample of tissue (biopsy), usually from the mucosa of the small intestine. To do this, your doctor will usually perform an upper endoscopy. The procedure uses a thin, flexible tube (endoscope) with a light and a built-in camera that passes through the mouth, the throat, the trachea, and stomach into the small intestine. The scope that allows your doctor to view your digestive passages and take tissue samples. During the procedure, doctors remove tissue samples from various sites in the small intestine. A doctor examines the tissue under a microscope in a laboratory. He or she looks for the presence of disease-causing bacteria and their sores (lesions), and in particular by Tropheryma whipplei bacteria. If these tissue samples does not confirm the diagnosis, your doctor may take a sample of tissue from an enlarged lymph node or to perform other tests. In some cases, your doctor may ask you to swallow a capsule that contains a small camera. The camera can take pictures of your digestive passages of his doctor to see. A DNA-based test known as polymerase chain reaction, which is available at some medical centers, you can detect Tropheryma whipplei bacteria in biopsy samples or cerebrospinal fluid samples.
  • Blood tests. Your doctor may also order blood tests, such as a complete blood count. Blood tests can detect certain conditions associated with Whipple's disease, in particular anemia, is a decrease in the number of red blood cells, and low concentrations of albumin, a protein in your blood.

The biopsy. An important step in the diagnosis of Whipple's disease is to take a sample of tissue (biopsy), usually from the mucosa of the small intestine. To do this, your doctor will usually perform an upper endoscopy. The procedure uses a thin, flexible tube (endoscope) with a light and a built-in camera that passes through the mouth, the throat, the trachea, and stomach into the small intestine. The scope that allows your doctor to view your digestive passages and take tissue samples.

During the procedure, doctors remove tissue samples from various sites in the small intestine. A doctor examines the tissue under a microscope in a laboratory. He or she looks for the presence of disease-causing bacteria and their sores (lesions), and in particular by Tropheryma whipplei bacteria. If these tissue samples does not confirm the diagnosis, your doctor may take a sample of tissue from an enlarged lymph node or to perform other tests.

In some cases, your doctor may ask you to swallow a capsule that contains a small camera. The camera can take pictures of your digestive passages of his doctor to see.

A DNA-based test known as polymerase chain reaction, which is available at some medical centers, you can detect Tropheryma whipplei bacteria in biopsy samples or cerebrospinal fluid samples.

Treatment

The treatment of Whipple's disease is with antibiotics, either alone or in combination, that can destroy the bacteria that cause the infection.

The treatment is long-term, usually lasting a year or two, with the objective of destroying the bacteria. But the relief of the symptoms usually comes much faster, often within the first week or two. Most people without a brain or the nervous system, the complications of making a full recovery after a full course of antibiotics.

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

Due to the prolonged use of antibiotics, your doctor should monitor your condition for the development of drug resistance. If relapse during treatment, your doctor may change your antibiotic.

The standard treatment for cases

In most cases, the therapy of Whipple's disease begins with two to four weeks of ceftriaxone or penicillin administered through a vein in your arm. Following the initial therapy, it is likely that you will have to 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, nausea, and vomiting.

Other medications that have been suggested as an alternative in some cases include oral doxycycline (Vibramycin, Doryx, others) in combination with the anti-malarial drugs, hydroxychloroquine (Plaquenil), which is likely to have to take one to two years.

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

The relief of the symptoms

Symptoms should improve within one to two weeks of starting antibiotic treatment, and completely disappears within about a month.

But despite the fact that the symptoms improve quickly, other laboratory tests may reveal the presence of bacteria during two or more years after starting to take antibiotics. Follow-up tests will help your doctor to determine when you can stop taking the antibiotics. Regular monitoring can also detect the development of resistance to a particular drug, often indicated by a lack of improvement in symptoms.

Even after a successful treatment, Whipple's disease can recur. Doctors usually advise regular check-ups. If you've experienced a recurrence, you'll need to repeat the therapy with antibiotics.

Take supplements

Because of the nutrients, the absorption of the difficulties associated with Whipple's disease, your doctor may recommend taking supplements of vitamins and minerals to ensure adequate nutrition. Your body may require a vitamin D supplement, folic acid, calcium, iron and magnesium.

Preparing for your appointment

If you have common signs and symptoms of Whipple's disease, make an appointment with your doctor. Whipple's disease is rare, and the signs and symptoms may indicate other, more common disorders, so it can be difficult 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 the treatment of the condition.

If your doctor is not sure about the diagnosis, he or she may refer you to a doctor who specializes in diseases of the digestive system, or to another specialist depending on the symptoms that you are having.

Here's some information to help you prepare for your appointment and know what to expect from your doctor.

Collection of information by advance

  • Write down your symptoms, even when he noticed for the first time and how they may have changed or worsened with time.
  • Write down your key medical information, including other conditions with which you have already been diagnosed and the names of all the medications, vitamins, and supplements you are taking.
  • Write down key personal information, including any recent changes or stressors in your life. These factors can be connected to a digestive system signs and symptoms.
  • Have a family member or friend along, if possible. Someone who accompanies you may remember something that you missed or forgot.
  • Write questions to ask their doctor. Creating your list of questions in advance can help you make the most of your time with your doctor.

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

  • What is the most likely cause of my condition?
  • There are other possible causes for my condition?
  • What diagnostic tests do I need?
  • What treatment approach do you recommend?
  • I have other medical conditions. How can I manage together?
  • How soon do you expect that my symptoms improve with treatment?
  • How long will I have to take medication?
  • Am I at risk of complications of this condition?
  • Am I at risk of a recurrence?
  • How often do you need to see me for the monitoring?
  • Do I have to change my diet?
  • Should I take nutritional supplements?
  • Are there lifestyle changes I can do to help control or reduce the symptoms?

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

What to expect from your doctor

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

  • What are your symptoms, and when they realized they?
  • The symptoms worsened over time?
  • Are their symptoms tend to get worse after a meal?
  • You have lost weight without trying?
  • Make your joint pain?
  • Do you feel weak or tired?
  • Do you have difficulty breathing or coughing?
  • Has developed confusion, or memory problems?
  • Have you noticed problems with your eyes or vision?
  • Has someone close to you has had similar signs or symptoms recently?
  • Have you been diagnosed with any other medical condition, including allergies to food?
  • Do you have any family history of diseases of the intestine or colon cancer?
  • What medications are you taking, including prescription and over-the-counter medications, vitamins, herbs and supplements?
  • Are you allergic to any medications?
Symptoms and treatment of Whipple's disease