Epidemiology of Whipple's Disease in the USA Between 2012 and 2017: A Population-Based National Study.

OBJECTIVE: Prior studies on the epidemiology of Whipple's disease are limited by small sample size and case series design. We sought to characterize the epidemiology of Whipple's disease in the USA utilizing a large population-based database. METHODS: We queried a commercial database (Explorys Inc, Cleveland, OH), an aggregate of electronic health record data from 26 major integrated healthcare systems in the USA. We identified a cohort of patients with a diagnosis of Whipple's disease based on systemized nomenclature of medical terminology (SNOMED CT) codes. We calculated the overall and age-, race-, ethnicity, and gender-based prevalence of Whipple's disease and prevalence of associated diagnoses using univariate analysis. RESULTS: A total of 35,838,070 individuals were active in the database between November 2012 and November 2017. Of these, 350 individuals had a SNOMED CT diagnosis of Whipple's disease, with an overall prevalence of 9.8 cases per 1 million. There was no difference in prevalence based on sex. However, prevalence of Whipple's disease was higher in Caucasians, non-Hispanics, and individuals > 65 years old. Individuals with a diagnosis of Whipple's disease were more likely to have associated diagnoses/findings of arthritis, CNS disease, endocarditis, diabetes, malignancy, dementia, vitamin D deficiency, iron deficiency, chemotherapy, weight loss, abdominal pain, and lymphadenopathy. CONCLUSIONS: To our knowledge, this is the largest study to date examining the epidemiology of Whipple's disease. In this large population-based study, the overall prevalence of Whipple's disease in the USA is 9.8 cases per 1 million people. It affects men and women at similar rates and is more common in Caucasians, non-Hispanics, and people > 65 years old.

Tropheryma whipplei aortic valve endocarditis, cured without surgical treatment.

BACKGROUND: Culture-negative endocarditis due to Tropheryma whipplei is a rare disease. Mostly the diagnosis is made by histologic examination of resected heart valve tissue. CASE PRESENTATION: In this case report, we described a patient with a classical Whipple's disease. Transesophageal echocardiography (TEE) showed a vegetation on noncoronary cusp of the aortic valve. Whipple's disease was confirmed by positive Tropheryma whipplei polymerase chain reaction (PCR) in EDTA blood and a duodenal biopsy with positive periodic acid-Schiff stain (PAS) macrophages. CONCLUSION: Due to timely diagnosis, our patient was treated with antibiotics without valve replacement.

Whipple's disease with destructive arthritis, abdominal lymphadenopathy, and central nervous system involvement.

We describe a patient with Whipple's disease who had an unusual erosive and destructive polyarthritis, massive abdominal lymphadenopathy, asymptomatic central nervous system involvement, and rare manifestations of orbital pseudotumor and orchitis with epididymitis. Taking oral therapy with trimethoprim-sulfamethoxazole he had recurrent flares of orbital pseudotumor, an episode of orchitis with epididymitis, and persistent polymerase chain reaction T. whipplei-positive cerebrospinal fluid. Resolution was achieved with a one month course of intravenous ceftriaxone and a 6 month course of azithromycin, and no relapse occurred during 24 months of followup.

Whipple's disease demonstrated by double contrast small bowel enema with barium and methylcellulose.

The radiologic findings on small bowel enema examination using barium and methylcellulose (SBE + Ba + MC) in a patient with Whipple's disease before and after treatment are described. The changes on SBE + Ba + MC corresponded well to the clinical and morphologic picture. The advantages of this double contrast technique compared to the enteroclysis with barium alone are demonstrated. The SBE + Ba + MC is a good method to demonstrate the manifestations of Whipple's disease involving the small bowel and the mesentery.