Whipple's disease, also known as intestinal lipodystrophy, is an extremely rare bacterial infection that typically affects the small intestine. It disrupts the normal digestive process, prevents the intestine from absorbing nutrients (malabsorption), and may cause immune system malfunctions. Left untreated, it can sometimes be fatal, but the condition can be cured with antibiotics.
Symptoms tend to develop slowly over the course of several years and typically include diarrhea, abdominal cramping, weight loss (due to the malabsorption of nutrients), loss of appetite, fatigue, weakness, fatty stools, joint inflammation, and fever.
Causes and Risk Factors
Whipple's disease is an infection by the bacterium Tropheryma whippelii (T. whippelii). T. whippelii causes lesions on the lining of the small intestine which damage the villi (tiny, finger-like structures that protrude from the intestine wall and aid in nutrient absorption).
Whipple's disease primarily affect men (over 80% of cases), and most commonly occurs during middle age. Rates of infection are higher for Caucasians than other ethnic populations in North America. Some researchers believe that a genetic defect may make some people more susceptible to infection by T. whippelii.
In addition to taking a medical history and discussing your symptoms, your physician will most likely do a tissue biopsy if Whipple's disease is suspected. Other diagnostic tools might include blood tests and fecal fat tests, which can detect symptoms that the infection is present.
Long-term antibiotic therapy is successful at eradicating T. whippelii infection and curing Whipple's disease. There are many different antibiotics that are effective in killing the bacteria, both alone and in combination. Your physician will determine what regime will work best for your case. Treatment generally will involve a hospital stay at first so that antibiotics can be administered intravenously, usually a 14-day course of treatment. Oral antibiotics will follow, and it may be necessary to take them for up to a year to ensure the infection is gone. Vitamin supplements and nonsteroidal anti-inflammatory medications may also be recommended to increase nutrient retention and lessen joint pain.
When your small intestine is unable to absorb nutrients such as vitamins, protein, sugars, or fats, this condition is referred to as malabsorption. This is a serious condition with possible long-term complications if left untreated.
In addition to weight and muscle loss, malabsorption may lead to diarrhea, bloating, cramping, frequent bulky stools, and a distended stomach. Children may exhibit a failure to thrive (significantly lower weight and height than peers in their age range).
Causes and Risk Factors
Many diseases and conditions can trigger malabsorption including:
- Chronic pancreatitis
- Whipple's disease
- Celiac disease
- Cystic fibrosis
- Bassen-Kornzweig syndrome
- Biliary atresia
- Lactose intolerance
- Milk protein intolerance (either cow's milk or soy milk)
- Pernicious anemia
- Certain parasites
Age can also be a factor in malabsorption; the production of digestive enzymes is sometimes slows in older adults, which can diminish the absorption of nutrients.
Diagnosis is generally made as a result of the presence of related symptoms and can be confirmed with blood and fecal fat tests. A small intestine biopsy or pancreatic function test may also be warranted to determine what might be causing the malabsorption, if not already known or suspected.
Treatment will depend on the underlying disease or condition that is triggering the malabsorption. Enzyme replacements, anti-diarrheals, and vitamin supplements may also be advised.
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