Epidemiology
A striking predilection for men of European ancestry was reported in a review of 664 patients with Whipple disease: 86% of patients were male, with 98% of white descent, and a mean age at diagnosis of 49 years.[14] Among patients mainly from the US, 35% were farmers, and 66% had occupational exposure to soil or animals. A consistent familial clustering was not found.
One large population-based study reported an overall prevalence of 9.8 cases per 1 million people in the US during the period 2012 to 2017.[19] In this study, Whipple disease prevalence did not differ with respect to sex (males 10.6 cases per 1 million; females 9.6 cases per million). Prevalence was greater in white people compared with African-Americans (13.9 cases per 1 million vs 7.6 cases per 1 million, respectively). Prevalence was 7.9 cases per 1 million in individuals ages 65 years and under, compared with 24.4 cases per 1 million in individuals ages over 65 years. Mean age at diagnosis could not be calculated because electronic health record data categorized individuals by age-group.[19] Another US-based study reported the prevalence of Whipple disease as 4.6 per one million hospitalizations during the period of 2016 to 2018.[20] In this study, a mean age of 60.2 ± 1.6 years was calculated for Whipple disease patients at diagnosis, and 67% of patients were male.
In Germany, annual incidence rates remained relatively stable among Whipple disease patients (n=110) diagnosed at 5 institutions during 1965-95.[21] However, mean age at diagnosis increased throughout the study time period: 1965-75, 48.7 years; 1976-85, 50.7 years; 1986-95, 57.0 years. An increasing proportion of women was also reported (1965-85, 4%; 1986-1995, 22%).[21]
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