Whipple's disease

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Classification according to ICD-10
K90.8 Other intestinal malabsorption
- Whipple's disease
M14.8 * Arthropathies in other specified diseases elsewhere classified
- arthritis in Whipple's disease
ICD-10 online (WHO version 2019)

The Whipple ( Whipple's disease , Whipple's disease , intestinal lipodystrophy ; English Whipple's disease ) is a rare bacterial infection of the small intestine caused by Tropheryma whipplei . The "classic" and unspecific symptoms are joint pain ( arthralgia ), abdominal pain and diarrhea .

history

Whipple's disease was first described in 1907 by George Hoyt Whipple (1878-1976), a pathologist from Rochester , New York State . He also described rod-shaped structures in lymph nodes as the cause, without being able to identify them. These were only recognized as bacilli in 1961 and identified as Tropheryma whipplei from the group of actinomycetes in 1991/1992 .

Epidemiology

While the bacterium Tropheryma whipplei is ubiquitous in nature, and studies have shown it to be in up to 20% of stool samples from healthy people, infection is very rare. The incidence is reported to be around one case per million people per year.

Pathogenesis (disease development)

The bacteria ( Tropheryma whipplei ) probably enter the stomach and upper part of the small intestine through oral ingestion . There they are phagocytosed by macrophages , remain in the mucous membrane ( mucosa ) and cause lymphatic congestion . As a result, the absorption of nutrients through the intestinal wall is inhibited and malabsorption syndrome occurs . Swollen intestinal villi, atrophic (thinned) epithelium and wide lymphatic vessels determine the histological picture. In the mucosa there are storage cells with lipid droplets and stainable bacteria in the cytoplasm . The storage cells (macrophages) appear bright red in the PAS color.

Symptoms

Intestinal symptoms

Extraintestinal symptoms

Diagnosis

Whipple's disease in the preparation of a biopsy

After the structures mentioned by Whipple in "intestinal lipodystrophy" were recognized as bacilli in 1961, the disease was diagnosed until 1992 exclusively through a biopsy (mucous membrane sample) with histopathological examination. Since the team headed by DA Relman at Stanford University in California was able to largely decipher the identity of the pathogen, which they named after Whipple, the diagnosis has also been made using the genetic detection of Tropheryma whipplei . Some of the infected small intestine cells mutate into SPC cells, Sickle Particle Containing Cells . Detection of Tropheryma whipplei from cerebrospinal fluid (nerve water) can be done by PCR. In addition, Whipple's disease shows an increase in inflammation levels (ESR, CRP), anemia ("anemia") and leukocytosis.

therapy

Classic antibiotics for the treatment of this bacterial infectious disease are or were chloramphenicol , penicillin , streptomycin and co- trimoxazole . An effective control of the extracerebral infection may be possible in this way; however, years later there may be another outbreak of the disease, this time originating in the brain.

The treatment regimen recommended in the literature is questioned by studies with regard to the antibiotics to be used and the duration of medication. The treatment of first choice is the administration of a broad-spectrum antibiotic (e.g. ceftriaxone ) for two weeks (alternatively meropenem ). This should be followed by maintenance therapy or relapse prevention with cotrimoxazole (1st choice) or tetracycline ( e.g. doxycycline ) for months up to two years.

In addition to this standard therapy, especially for endocarditis, the combination of ceftriaxone and meropenem, followed by cotrimoxazole according to European recommendations, can be considered. According to the AWMF, if the central nervous system is affected, treatment should be given with penicillin or ceftriaxone combined with streptomycin, followed by cotrimoxazole.

In addition, there should be adequate substitution of electrolytes, vitamins and trace elements. The success of the therapy can be monitored by control endoscopies.

forecast

In most cases, Whipple's disease is fatal if left untreated.

literature

  • Desnues B, Ihrig M, Raoult D, Mege JL: Whipple's disease: a macrophage disease . In: Clin Vaccine Immunol. , 2006 Feb, 13 (2), pp. 170-178. Review, PMID 16467322 .
  • F Dutly, M Altwegg: Whipple's disease and “Tropheryma whippelii” . In: Clin Microbiol Rev. , 2001 Jul, 14 (3), pp. 561-583. Review, PMID 11432814 .
  • F Fenollar, D Raoult: Whipple's disease . In: Clin Diagn Lab Immunol. , 2001 Jan, 8 (1), pp. 1-8. Review, PMID 11139188 .
  • DA Relmann et al .: Identification of the uncultured bacillus of Whipple's disease. In: New England Journal of Medicine. Volume 327, 1992, pp. 293-301.

Web links

Individual evidence

  1. ^ GH Whipple: A retrospectively undescribed disease characterized anatomically by deposits of fat and fatty acids in the intestinal and mesenteric lymphatic tissues. In: Bulletin Johns Hopkins Hosp. 18, 1907, pp. 382-391.
  2. ↑ Camila D. Odio, Corey R. O'Brien, Jeremy Jacox, Dhanpat Jain, Alfred I. Lee: Clinical Problem-Solving: Cryptic Cachexia. In: New England Journal of Medicine . Volume 383, No. 1, July 2, 2020, Volume 383, pp. 68-74. DOI: 10.1056 / NEJMcps1817531 .
  3. ↑ Wilfried Obst, Ulrike von Arnim, Peter Malfertheiner: Whipple's Disease . In: Visceral Medicine , June 30, 2014, PMC 4513828 (free full text) (English)
  4. ↑ Jean-christophe Lagier, Hubert Lepidi, Didier Raoult, Florence Fenollar: Systemic: Clinical Presentation of 142 Patients With Infections Diagnosed or Confirmed in a Reference Centertropheryma whipplei: Clinical Presentation of 142 Patients With Infections Diagnosed or Confirmed in a Reference Center . In: Medicine . tape 89 , no. 5 , September 1, 2010, ISSN  0025-7974 , p. 337-345 , doi : 10.1097 / MD.0b013e3181f204a8 ( ovid.com [accessed January 6, 2019]).
  5. ↑ Ch. R .: Identification of the bacterium causing Whipple's disease. In: Journal of General Practice. Volume 68, No. 29, October 1992, p. 11.
  6. ↑ jac.oxfordjournals.org
  7. ↑ journals.lww.com
  8. ↑ Atypical pathogen-related meningoencephalitis. 2008.
  9. ^ Marianne Abele-Horn: Antimicrobial Therapy. Decision support for the treatment and prophylaxis of infectious diseases. With the collaboration of Werner Heinz, Hartwig Klinker, Johann Schurz and August Stich, 2nd, revised and expanded edition. Peter Wiehl, Marburg 2009, ISBN 978-3-927219-14-4 , p. 259.