Whipples disease is a very rare bacterial infektion affecting the small bowel. The infection is caused by a bacteria Trophynema whippelii. The infection can affect other organs than the bowel, e.g. the nervous system, eyes and the heart.
Typical symptoms caused by the infection in the bowel are diarrhea, stomach pain and malabsorption (causing weight loss, anaemia and hypoalbuminemia). If the infection has spread to other organs the patient might suffer from joint pain resembling rheumatoid arthritis, chronic cough, chest pain, heart failure, fever, fatigue and weakness. Typical neurologic symptoms are visual disturbances, headache, walking difficulties, memory loss, confusion and even dementia - and these symptoms can mimic many neurological diseases.
The symtoms develop very slowly over months or even years.
Whipples disease was described in 1907 by Georg Hoyt Whipple (1878-1976), an american physician. He called this disease lipodystrophia intestinalis, but he was aware of the probable bacterial infection as the cause of this disease. Prof Whipple got the Nobel Prize in 1934 as a reward of his studies on pernicious anaemia.
It took nearly one hundred years until the bacteria, Tropheryma whipplei, was identified and cultivated. It is not known how this infection is aquired, no person-to-person transmission has been dicumented.
The typical patient is a caucasian male between 40 and 70 years old. The disease can be readily visible during an upper endoscopy, as in these images. Duodenal biopsies give the diagnosis, showing foamy macrophages which contain the bacteria, easily stained by PAS stain. A PCR examination can be made, but apparently there are many symtomfree healthy carriers of this bacteria.
This disease had often a fatal outcome until it was discovered that treatment with tetracycline was effective. The treatment should go on for a long time, one to two years, to avoid recurrence. In severe cases intravenous antibiotic therapy for two weeks is often used. Trimetoprim-sulfa is an effective alternative to tetracycline. The symptom resolve quite soon after starting therapy - in a few weeks, and recovery from this serious disease is usually uneventful if severe neuologic symtoms has not developed.
Whipples disease is truely very rare, affecting perhaps one in a million people. The diagnose is easy - small bowel biopsies taken during an upper endoscopy will usually give a firm diagnose.