Whipple's Disease Health Article

Definition

Whipple's disease is a rare infectious disorder that can affect many areas of the body, including the gastrointestinal and central nervous systems. Caused by the bacteria Tropheryma whipplei, it is typically diagnosed from malabsorption symptoms such as diarrhea and weight loss. If the central nervous system is infected, Whipple's disease can cause impairment of mental faculties and lead to dementia. It can be treated successfully with antibiotic therapy, but up to a third of patients suffer relapse.

Description

Whipple's disease, also known as intestinal lipodystrophy, was first reported in 1907 by George Hoyt Whipple (1878–1976). An autopsy on a thirty-seven year old male missionary revealed a granular accumulation of fatty acids in the walls of the small intestine and lymph nodes.

Historically, Whipple's disease has been considered an gastro-intestinal disorder, however, in the 1960s it was realized that other organs could be involved, with or without intestinal infection. It is now considered a systemic infection with a wide range of possible symptoms.

Demographics

The disorder typically affects middle-aged men of European descent. Most cases have been reported in North America and Europe. Many texts suggest the disorder affects eight times as many males as females, although there is some evidence to suggest the rate in females is rising.

The disease is extremely rare and no reliable estimate of incidence is known. Farmers and other rural people are most often diagnosed with Whipple's disease, but as yet, no specific environmental factors have been linked to the disorder.

Causes and symptoms

The bacterium that causes Whipple's disease was only successfully cultured in 1997. Tropheryma whipplei belongs to the high G+C phylum of gram-positive bacteria, and its genome was sequenced in 2003.

Whipple's disease has traditionally been regarded as a malabsorption disease of the small intestine, but in most cases the first symptoms are arthritic joints, which can precede the malabsorption symptoms of Whipple's disease by many years. Commonly, the disease progresses to the small intestine. Symptoms then include diarrhea, anemia, weight loss, and there is often fat present in the stool, all due to the bacteria disrupting absorption of fat and nutrients. If untreated, other malabsorption problems, such as reductions in the levels of calcium and magnesium, may result. Fever and night sweats are common, as well as general weakness. There are many further possible symptoms depending on the organs affected.

In cases where the central nervous system is affected, there may be a decrease in intellectual abilities, insomnia, hearing loss or tinnitus (ringing in the ears), and uncontrolled muscle movements (ataxia) or eye movements. If untreated, the disorder can lead to dementia and progressive brain cell death, leading to coma and death over a period of months to years.