Toward a new understanding of Whipple’s disease

Flemmer, Mark; Flenner, Ronald W.

doi:10.1007/s11894-000-0022-z

Cited by 3 publications

(1 citation statement)

References 25 publications

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“…In our cohort, SB PAS stain and PCR were positive in 14% and 42%, respectively, of LWD cases without weight loss or chronic diarrhea. These results are congruent with previous studies demonstrating increased sensitivity of duodenal PCR compared with PAS staining [ 11 , 16 , 30 ]. Lagier and colleagues recently reported 24 cases of LWD in which duodenal tissue PAS stain and PCR were positive, 0% (0/22) and 6.7% (1/15), respectively [ 1 ].…”

Section: Discussionsupporting

confidence: 93%

Diagnostic Approach for Classic Compared With Localized Whipple Disease

Crews

Cawcutt

Pritt

et al. 2018

Open Forum Infectious Diseases

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BackgroundWhipple disease (WD), a rare systemic infection caused by Tropheryma whipplei, can be a diagnostic challenge due to its variable presentation. The role of T. whipplei polymerase chain reaction (PCR) is unclear as small bowel biopsy with Periodic acid-Schiff (PAS) staining remains the diagnostic gold standard. Individualized diagnostic approaches based on variable clinical manifestations are underutilized. We investigated the methodologies employed at our institution to diagnose WD.MethodsWe retrospectively collected all cases of WD diagnosed from 1994 to 2016. Microbiology laboratory and anatomic pathology databases were queried. Case characteristics and disease clinical phenotypes (classical, localized WD arthritis, and localized central nervous system [CNS] disease) were described. The diagnostic approach and testing yield were analyzed and reported.ResultsThirty-three cases of WD were diagnosed (18 classic WD [CWD], 9 localized WD arthritis [LWD], 6 CNS WD). Misdiagnosis and delay in diagnosis were frequent. Diagnostic approach and test yield differed by classical vs localized WD involvement. Small bowel tissue biopsy PAS stain/PCR was overwhelmingly positive (86%/92%) in CWD, yet seldom positive (12%/42%) in LWD (P < .001). Affected joint synovial fluid PCR was frequently positive in both CWD (100%, 3/3) and LWD (85%, 6/7).ConclusionsThese results support the role of small bowel biopsy PAS stain/PCR in the diagnosis of CW, though this approach may be of limited utility in LWD or CNS WD without gastrointestinal symptoms. Affected joint synovial fluid or cerebrospinal fluid PCR was frequently positive in both CWD and LWD, supporting its diagnostic usefulness.

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