Whipple's disease

The Whipple ( Whipple 's disease, intestinal lipodystrophy; engl. Whipple 's disease, first described in 1907 by, and named after George H. Whipple, 1878-1976, a pathologist from Rochester ) is a rare disorder of the small intestine. It is caused by the 1991/1992 identified bacterium Tropheryma whipplei from the group of actinomycetes. Since 2003, the complete genome of the bacterium has been sequenced and analyzed. Preference is given to affected men ( Androtropie ), children and adolescents, however, are not between 30 and 60 years of age apparently. Also noticeable is a socialized with the disease immunodeficiency, which is not clear whether this cause or consequence of the infection.

Pathogenesis ( disease development )

Probably go on acceptance by ingestion, the bacteria ( Tropheryma whipplei ) in the stomach and upper small intestine. There they are phagocytosed by macrophages, remain lying in the mucous membrane ( mucosa ) and cause lymphatic congestion. Characterized nutrient uptake is inhibited by the intestinal wall and it comes to malabsorption syndrome. Swollen villi, atrophic ( diluted ) epithelium and large lymphatics determine the histological picture. In the mucosa are memory cells with lipid droplets and stainable bacteria in the cytoplasm. The memory cells ( macrophages) appear bright red in PAS staining.

Symptoms

Intestinal symptoms

  • Typical symptoms of malabsorption with weight loss and foul-smelling chairs
  • Abdominal pain
  • (Diarrhea ) and steatorrhea diarrhea ( steatorrhea )
  • Weight loss as a result of diarrhea
  • From the intestine of a lymphatic and vascular spread usually takes place in parallel

Extra-intestinal symptoms

  • Enteropathic arthritis / spondyloarthropathy / sacroiliitis (most common first symptom ) 18%
  • Microcytic hypochromic anemia (iron deficiency )
  • Thus the typical brownish- pale skin color
  • Fever
  • Lymphadenopathy, especially the mesenteric lymph nodes to swell
  • Rare cardiac involvement and possible endocarditis, heart failure
  • CNS involvement ( dementia)
  • Ocular involvement (eg, gaze palsy or uveitis )
  • Polyserositis
  • Promovierend associated with the bacterial infection is an HLA -B27 -associated immunodeficiency likely.

Diagnostics

  • Until 1992, only a biopsy ( mucosal sample) with histopathological examination
  • Gene detection of Tropheryma whipplei today
  • The infected small intestine cells mutate partially SPC cells, Sickle Particle Containing Cells
  • Tropheryma whipplei from the detection of cerebrospinal fluid ( cerebrospinal fluid ) by PCR
  • Increase in inflammatory markers ( ESR, CRP )
  • Anemia (anemia ), leukocytosis
  • 10-20% of patients with ulcerative colitis or Crohn's disease also develop a Whipple.

Therapy

Traditional antibiotics for the treatment of these bacterial infectious disease are or were chloramphenicol, penicillin, streptomycin, and cotrimoxazole. This uses may be possible to effectively combat the extracerebral infection; nevertheless, it may be years later to a renewed, this time emanating from the brain disease outbreak.

The recommended treatment regimen in the literature is provided regarding the asset to be used antibiotics and medication duration by studies in question. Previously regarded as first-line therapy, the administration of a broad-spectrum antibiotic liquorgängigen (eg ceftriaxone ) over two weeks. Because maintenance therapy or relapse prophylaxis with cotrimoxazole (1st choice) or tetracycline for months to two years should be connected.

In addition, an adequate replacement of electrolytes, vitamins and trace elements should be. The success of treatment can be monitored by follow-up endoscopy.

Forecast

In most cases of Whipple's disease is fatal without treatment.

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