Whipple’s disease-colloquially known as Whipple’s disease-(synonyms: Intestinal lipodystrophy; Lipodystrophia intestinalis; Whipple’s disease; ICD-10-GM K90.8: Other intestinal malabsorption) is a chronic infectious disease caused by the actinomycete (group of bacteria) Tropheryma whippelii (gram-positive rod bacterium) that affects the small intestine. In addition to the intestinal system, other organ systems may also be affected (multisystem disease).
The pathogen is found, for example, in sewage.
The disease occurs very rarely.
Human-to-human transmission: No.
Sex ratio: Males to females is 3: 1. Previous studies have reported a ratio as high as 8: 1.
Peak incidence: The disease occurs predominantly between the ages of 30 and 60. At diagnosis, affected individuals are on average 55 years old.
More precise data on prevalence (frequency of disease) and incidence (frequency of new cases) are not available. In Central Europe, it appears to affect less than one person per 1 million citizens per year. It is estimated that asymptomatic colonization of the intestinal lumen is present in 2-4% of the population (in Europe).
Course and prognosis: In addition to the intestinal system, other organ systems may also be affected (multisystem disease). Often, the first symptom of Whipple’s disease is oligoarthritis (occurrence of joint inflammation (arthritis) in fewer than 5 joints) or sacroiliitis (inflammation of the sacroiliac joint between the sacrum and the ilium). These symptoms may precede the intestinal symptoms – such as diarrhea (diarrhea), steatorrhea (fatty stools) – by up to 10 years.A cure is possible only by eradication of the pathogen (elimination of the germ). If left untreated, the disease is lethal.
Whipple’s disease can be recurrent. Because the course of this rare disease is not precisely known, regular follow-up over an extended period of time should occur.
