Intestinal lipodystrophy

Definition

Whipple's disease is a rare condition in which infection makes the intestine unable to properly absorb nutrients. This is called malabsorption.

Alternative Names

Causes

Whipple's disease is most likely caused by infection with Tropheryma whippelii. The disorder primarily affects middle-aged white men, and the onset of symptoms is usually slow. Without treatment, it may be fatal.

Whipple's disease is extremely rare, and risk factors are unknown.

Symptoms

• Abdominal pain
• Fatty stools
• Fever
• Diarrhea
• Weight loss
• Gastrointestinal bleeding
• Enlarged lymph glands
• Joint pain
• Edema
• Gray to brown skin coloration
• Mental status changes
• Memory loss

Exams and Tests

Primary tests:

• Small bowel biopsy showing infection-fighting cells containing bacteria (other body parts can also be biopsied)
• Complete blood count (CBC) showing anemia

• Fecal fat
• D-xylose absorption
• Albumin

Treatment

A person with Whipple's disease needs long-term antibiotic therapy to treat infections of the brain and central nervous system. A medicine called ceftriaxone is given through a vein (IV), followed by an antibiotic taken by mouth, such as trimethoprim -sulfamethoxazole. This antibiotic is taken for up to one year.

If symptoms come back during antibiotic use, the antibiotic or antibiotics may be changed.

Because signs of the disease can return after completing therapy, the patient should be closely followed by a doctor. Those who have nutritional deficiencies due to malabsorption will need dietary supplements.

Outlook (Prognosis)

Without treatment, the condition is usually fatal. Treatment improves the chance of a good outcome.

Possible Complications

• Nutritional deficiencies
• Weight loss
• Reappearance of symptoms, suggesting drug resistance

When to Contact a Medical Professional

Call your health care provider if you have persistent abdominal pain and diarrhea.

If you are being treated for Whipple's disease, call your health care provider if symptoms worsen or do not improve, if symptoms reappear, or if new symptoms develop.