Isabel A Hujoel1, David H Johnson2, Benjamin Lebwohl3, Daniel Leffler4, Sonia Kupfer5, Tsung-Teh Wu6, Joseph A Murray2, Alberto Rubio-Tapia7. 1. Division of Internal Medicine, Mayo Clinic, Rochester, MN, USA. 2. Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street, S.W., Rochester, MN, 55905, USA. 3. Division of Digestive and Liver Diseases, Department of Medicine, Columbia University, New York, NY, USA. 4. Division of Gastroenterology and Hepatology, Beth Israel Deaconess Medical Center, Boston, MA, USA. 5. Division of Gastroenterology and Hepatology, University of Chicago, Chicago, IL, USA. 6. Division of Anatomic Pathology, Mayo Clinic, Rochester, MN, USA. 7. Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street, S.W., Rochester, MN, 55905, USA. rubiotapia.alberto@mayo.edu.
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.
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 TWinfection. METHODS: Cases of TWinfection 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:TWinfection can present as intestinal or localized disease. Negative small bowel PAS and PCR do not exclude the diagnosis of TWinfection, and blood PCR is insensitive for active infection.
Authors: Wenjun Li; Florence Fenollar; Jean-Marc Rolain; Pierre-Edouard Fournier; Gerhard E Feurle; Christian Müller; Verena Moos; Thomas Marth; Martin Altwegg; Romana C Calligaris-Maibach; Thomas Schneider; Federico Biagi; Bernard La Scola; Didier Raoult Journal: Microbiology Date: 2008-02 Impact factor: 2.777