Whipple disease is a rare infectious multisystem disorder caused by the actinobacteria Tropheryma whipplei.
The incidence of Whipple disease is not truly known, one Swiss study estimated it at approximately 1 per 1.5 million per year 7.
The peak age for presentation is in the fifth decade of life. Caucasians are most often affected, and men are affected eight times more commonly than women 5. The disease is more common in farmers and those who work with soil and livestock 8.
Patients often present with a non-destructive migratory arthritis, weight loss, diarrhoea and abdominal pain. Less commonly patients present with fever, lymphadenopathy or hepatosplenomegaly 5. The presence of arthritis may precede other symptoms by years 8.
A suspected diagnosis of Whipple disease can be confirmed by showing periodic acid-Schiff-positive granular foamy macrophages from sampled tissue, such as the small bowel or a peripheral lymph node 5.
The small bowel (intestinal lipodystrophy) is a classical location although the disease can affect a multitude of other organ systems with or without small bowel involvement. Other locations include 1,2:
- central nervous system
These largely depend on the organ system involved:
- gastrointestinal manifestations of Whipple disease
- CNS manifestations of Whipple disease
- thoracic manifestations of Whipple disease
- cutaneous manifestations of Whipple disease
- joint manifestations of Whipple disease
Treatment and prognosis
CNS involvement with Whipple disease carries poor prognosis and it is invariably fatal without treatment. Approximately half of the patients may show some symptomatic improvement during antibiotic treatment. ~17.5% (range 2-33%) patients may relapse. Thus, early diagnosis and treatment are paramount for survival 6.
History and etymology
First described by George Hoyt Whipple, American (US) pathologist (1878-1976) in 1907 4.
- 1. Samuels T, Hamilton P, Shaw P. Whipple disease of the mediastinum. AJR Am J Roentgenol. 1990;154 (6): 1187-8. AJR Am J Roentgenol (citation) - Pubmed citation
- 2. Schnider P, Trattnig S, Kollegger H et-al. MR of cerebral Whipple disease. AJNR Am J Neuroradiol. 16 (6): 1328-9. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 3. Ledermann HP, Börner N, Strunk H et-al. Bowel wall thickening on transabdominal sonography. AJR Am J Roentgenol. 2000;174 (1): 107-17. AJR Am J Roentgenol (full text) - Pubmed citation
- 4. Whipple GH "A hitherto undescribed disease characterized anatomically by deposits of fat and fatty acid in the intestinal and mesenteric lymphatic tissues". Bull Johns Hopkins Hosp 18: 382–93. (1907)4
- 5. Haaga JR, Boll D. CT and MRI of the whole body. Mosby. (2009) ISBN:0323053750. Read it at Google Books - Find it at Amazon
- 6. Black DF, Aksamit AJ, Morris JM. MR imaging of central nervous system Whipple disease: a 15-year review. AJNR Am J Neuroradiol. 2010;31 (8): 1493-7. doi:10.3174/ajnr.A2089 - Pubmed citation
- 7. Dutly F, Altwegg M. Whipple's disease and "Tropheryma whippelii". Clin. Microbiol. Rev. 2001;14 (3): 561-83. doi:10.1128/CMR.14.3.561-583.2001 - Free text at pubmed - Pubmed citation
- 8. Bureš, Jan, Kopáčová, Marcela, Douda, Tomáš, Bártová, Jolana, Tomš, Jan, Rejchrt, Stanislav, Tachecí, Ilja. Whipple’s Disease: Our Own Experience and Review of the Literature. (2018) Gastroenterology Research and Practice. 2013: 478349. doi:10.1155/2013/478349 - Pubmed