Intestinal lipodystrophy is also known as Whipple’s disease. The medical eponym was named after George Hoyt Whipple, an American pathologist, who first described the intestinal lipodystrophy. Whipple’s disease is a disorder that affects multiple systems. It is rare and is seen in less than one per 1,000,000 people each year.
The condition results from a bacterial infection that causes inadequate absorption of the nutrients from your small intestine. Initially, only the small intestine is affected, but in later stages, the disease affects various parts of your body.
The bacterium that is responsible for Whipple’s disease is Tropheryma whipplei. The bacterium affects the inner lining of your small intestine and leads to the development of lesions and thickens the mucosal lining. The inner lining of small intestine consists of finger-like structures called villi that help to absorb the nutrients. Thickening of the villi leads to abnormal absorption of nutrients from the digested food in the small intestine. Thus, inadequate absorption of nutrients in the small intestine causes the symptoms of Whipple’s disease.
Additionally, many research studies suggest that a genetic defect in the functioning of the immune system makes an individual more susceptible to the disease.
Due to inadequate absorption of nutrients, different parts of your body are affected. The infection may spread from the digestive tract to other parts such as heart, lungs, brain, joints, and eyes. The common signs and symptoms of Whipple’s disease include the following:
The less common signs and symptoms of intestinal lipodystrophy include:
The risk of Whipple’s disease is high in males than females and is in the ratio of 4:1. The symptoms may begin between the ages 40 and 80. Whites in North America and Europe are more susceptible to the disease when compared with other races. Poor sanitary conditions and unhealthy food habits may also increase the risk of Whipple’s disease.
Intestinal lipodystrophy is a progressive disease that may become fatal in advanced stages. If the Whipple’s disease is left untreated, the disease may spread to the central nervous system and leads to irreversible damage and death.
Your doctor begins the diagnosis by thorough physical examination which includes checking for signs and symptoms of Whipple’s disease. Your doctor might order for the following tests to confirm the diagnosis.
Blood tests:A complete blood count may be recommended to determine the conditions associated with Whipple’s disease such as anemia.
Endoscopy: During the endoscope, a small flexible tube with a camera attached to it is inserted into your small intestine. This provides a complete view of your small intestine and helps to identify the presence of lesions.
Biopsy: A sample tissue of your intestinal wall is removed to examine for the presence of Tropheryma whipplei bacteria.
Generally, the treatment for Whipple’s disease is aggressive and lasts for one or two years to completely eradicate the causative organism. Antibiotics are recommended for the treating Whipple’s disease. Continuous monitoring may be required to check for the development of resistance to the drugs.
Your doctor begins the treatment with two to four weeks of intravenous (IV) ceftriaxone, followed by an oral dose of sulfamethoxazole-trimethoprim for one or two years.
If the infection is spread to the central nervous system, your doctor might prescribe an oral dose of doxycycline and hydroxychloroquine for 12 to 18 months.
The other treatment options in addition to antibiotics include the following:
The symptoms are relieved within one month of the antibiotic treatment. Continuing to take the antibiotics as prescribed will help to eradicate the disease completely.