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Case 229

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Forty-six-year-old male patient, socioeconomically deprived, living in an area of frequent flooding, was attended at the Alfa Institute of Gastroenterology HC-UFMG with dyspeptic symptoms, low fever, unintentional weight loss of 13 kg and intermittent diarrhea without abnormal elements in the stool, for the last five months. Also reported migratory polyarthralgia for about five years. His twin brother (monozygotic) had the same clinical picture. The additional investigation attached was requested.

Considering the clinical and epidemiological picture and complementary investigation results presented, which is the correct diagnosis?

a) Celiac disease

25%

b) Crohn's disease

25%

c) Whipple's disease

25%

d) Intestinal tuberculosis

25%
   

Image analysis

Image 1: endoscopic view of the beginning of the second portion of the duodenum presenting whitish areas (highlighted in green) interspersed with normal looking mucosa (in white).

 

Image 2: endoscopic view of the second portion of the distal duodenum showing extensive whitish areas. This finding is suggestive of villi dilation, lipid accumulation and lymphatic vessels ectasia. There are also thickening of the duodenal folds and edema of the mucosa causing reduction in the lumen. These findings, although quite specific for the diagnosis of Whipple's disease are not very sensitive, being observed only in 26% of cases.

 

Image 3: Histological section of duodenal mucosa showing significant enlargement of the villi due to edema (highlighted in blue) and the presence of hystiocytic infiltrate (part of which is highlighted in red), where we observe the accumulation of PAS-positive material. Note also, in the lamina propria, large empty spaces (in green), well-defined, whose limpid content has been removed for histological processing.

Image 4: description in details of the prior histologic slice showing the PAS-positive granulation within macrophages (in green), which correspond to the presence of Tropheryma whippleiviable bacilli, the intracellular bacteria that causes Whipple's disease.

Highlights

- Tropheryma whipplei is a commensal bacteria, relatively common, capable of causing a rare disease in susceptible individuals;

- The classic Whipple's disease is a form of disseminated infection and  it commonly presents as a triad of polyarthralgia, weight loss and diarrhea;

- The disease may present as a chronic or as an isolated form, affecting a single organ in the form of encephalitis or endocarditis;

- The diagnosis is made by the biopsy of the affected organ and coloring material through PAS and, in doubtful cases, PCR and / or immunohistochemistry can be used to confirm it;

- The treatment is done with long-term antibiotic therapy and relapses are frequent and sometimes late, requiring appropriate follow-up.

References

-Fenollar F, Lagier JC, Raoult D. Tropheryma whipplei and Whipple's disease.J Infect 2014 Aug;69(2):103-12.

- Apstein MD, Schneider T. Whipple's disease. UpToDate [internet] 2015 [acesso em Mar2016]. Available in: http://www.uptodate.com/contents/whipples-disease

- Mönkemuller K, Fry Lc, von Armin U, Neumann H, Evert M, Malfertheiner P. Whipple’s disease: an endoscopic and histologic study. Digestion 2008;77(3-4)

- Günter U, Moos V, Offenmüller G, Oelkers G, Heise W, Moter A, Loddenkemper C, Schneider T. Gastrointestinal diagnosis of classical Whipple disease: clinical, endoscopic, and histopatologic features in 191 patients. Medicine (Baltimore) 2015 Apr;94(15):e714.

- Ferrari Mde L, Vilela EG, Faria LC, Couto CA, Salgado CJ, Leite VR, Brasileiro Filho G, Bambirra EA, Mendes CM, Carvalho Sde C, de Oliveira CA, da Cunha AS. Whipple’s disease. Report of five cases with different clinical features. Rev Inst Med Trop São Paulo 2001 Jan-Feb;43(1):45-50.

Author

André Ribeiro Guimarães, 6th year medical student at Universidade Federal de Minas Gerais.

E-mail: guimaraesandrer[at]hotmail.com

Supervisors

Professor and Doctor Luciana Dias Moretzsohn, medical gastroenterologist and endoscopist, associated professor of the Department of Medical Clinic of Universidade Federal de Minas Gerias.

E-mail: ludias18[at]gmail.com

Reviewers

Bárbara Bragáglia, Débora Faria, Luísa Bernardino, Fellype Borges, Luanna Monteiro and Professor and Doctor Viviane Parisotto

Translated by 

Laio Bastos de Paiva Raspante, 4th year medical student at UFMG.

E-mail: laioopaiva[at]gmail.com

Test question

a)

25%

b)

25%

c)

25%

d)

25%

e)

25%
   

Commentics

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