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Tropheryma whipplei Infection (Whipple Disease) in the USA

  • Isabel A. Hujoel
  • David H. Johnson
  • Benjamin Lebwohl
  • Daniel Leffler
  • Sonia Kupfer
  • Tsung-Teh Wu
  • Joseph A. Murray
  • Alberto Rubio-Tapia
Original Article
  • 199 Downloads

Abstract

Background

Whipple disease (WD) is an infection caused by the bacterium Tropheryma whipplei (TW). Few cases have been reported in the USA.

Aims

To report on the demographics, clinical manifestations, diagnostic findings, treatment, and outcomes of TW infection.

Methods

Cases of TW infection diagnosed from 1995 to 2010 were identified in three US referral centers and from 1995 to 2015 in one. Definite classic WD was defined by positive periodic acid-Schiff (PAS) staining and probable WD by specific positive TW polymerase chain reaction (PCR) of intestinal specimens. Localized infections were defined by a positive TW PCR result from samples of other tissues/body fluids.

Results

Among the 33 cases of TW infections, 27 (82%) were male. Median age at diagnosis was 53 years (range 11–75). Diagnosis was supported by a positive TW PCR in 29 (88%) and/or a positive PAS in 16 (48%) patients. Classic WD was the most frequent presentation (n = 18, 55%), with 14 definite and 4 probable cases. Localized infections (n = 15, 45%) affected the central nervous system (n = 7), joints (n = 4), heart (n = 2), eye (n = 1), and skeletal muscle (n = 1). Blood PCR was negative in 9 of 17 (53%) cases at diagnosis. Ceftriaxone intravenously followed by trimethoprim and sulfamethoxazole orally was the most common regimen (n = 23, 70%). Antibiotic therapy resulted in clinical response in 24 (73%).

Conclusions

TW infection can present as intestinal or localized disease. Negative small bowel PAS and PCR do not exclude the diagnosis of TW infection, and blood PCR is insensitive for active infection.

Keywords

Whipple disease Intestinal lipodystrophy Tropheryma Malabsorption syndromes 

Notes

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflicts of interest.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Isabel A. Hujoel
    • 1
  • David H. Johnson
    • 2
  • Benjamin Lebwohl
    • 3
  • Daniel Leffler
    • 4
  • Sonia Kupfer
    • 5
  • Tsung-Teh Wu
    • 6
  • Joseph A. Murray
    • 2
  • Alberto Rubio-Tapia
    • 2
  1. 1.Division of Internal MedicineMayo ClinicRochesterUSA
  2. 2.Division of Gastroenterology and HepatologyMayo ClinicRochesterUSA
  3. 3.Division of Digestive and Liver Diseases, Department of MedicineColumbia UniversityNew YorkUSA
  4. 4.Division of Gastroenterology and HepatologyBeth Israel Deaconess Medical CenterBostonUSA
  5. 5.Division of Gastroenterology and HepatologyUniversity of ChicagoChicagoUSA
  6. 6.Division of Anatomic PathologyMayo ClinicRochesterUSA

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