Materiały źródłowe

• #1 The Epidemiology of Whipple’s Disease in the United States between 2012 and 2017: A Population based National Study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC6499665/

  Prior studies on the epidemiology of Whipples disease are limited by small sample size and case series design. We sought to characterize the epidemiology of Whipples disease in the United States utilizing a large population-based database. 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 Whipples disease, with an overall prevalence of 9.8 cases per 1 million. There was no difference in prevalence based on sex. However, prevalence of Whipples disease was higher in Caucasians, non-Hispanics, and individuals 65 years old. To our knowledge, this is the largest study to date examining the epidemiology of Whipples disease. In this large population-based study, the overall prevalence of Whipples disease in the US 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. In this study, we evaluated the prevalence of Whipples disease over 5 years in the Explorys database between 2012 and 2017. To our knowledge, this is the largest study to date that estimates the prevalence of this condition in the United States at the national level. This is also the first large study to describe race-, age- and sex-based prevalence of Whipples disease in the United States from national-level data. In the current study, we found that Whipples disease affects men and women at similar rates. This finding is in contrast to the widely held belief that Whipples disease mainly affects men. In the current study, older age was associated with an increased risk of Whipples disease. Prevalence was 7.9 cases per 1 million in individuals 65 years old compared to 24.4 cases per 1 million in individuals 65 years old. While we did not look at region-wise burden of Whipples disease, it is safe to assume that regional differences in gender, age and race distribution cannot alone account for our findings of Caucasian, non-Hispanic and elderly (65 years) predominance of Whipples disease. In summary, the analysis of the only population based national sample of Whipples disease cases thus far, from the large commercial database Explorys, estimates the overall prevalence of Whipples disease at 9.8/1,000,000. This estimate is larger than what has previously been extrapolated from case series. Consistent with prior case series data, we found that the prevalence of Whipples disease is higher in Caucasians as compared to African Americans. In contrast to prior data, though, we found that the prevalence of Whipples disease is similar between males and females and more common in individuals 65 years old.

• #2 An unusual presentation of Whipples disease: adenopathies, polyarthralgia and dermatomyositis-like symptoms

  https://www.explorationpub.com/Journals/em/Article/1001113

  Whipples disease (WD) is a very rare, chronic, systemic infectious disease with an estimated annual incidence of 3 in one million, which may be fatal if not diagnosed and treated appropriately. […] In a large population-based study conducted in the US, the overall prevalence of WD was 9.8 cases per 1 million people. […] The reservoir, route of transmission and natural occurrence is not identified and until now under research. […] According to literature, the most common host of this bacterium is the mucosa of the small intestine in human. […] Polymerase chain reaction (PCR) is increasingly used to identify bacterial 16S rDNA molecules and other targeted repeated sequences of Tropheryma whipplei (T. whipplei, formerly Tropheryma whippelii). […] The definitive diagnosis is established in PAS-positive histologic findings along with positive PCR.

• #3 Whipple Disease: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/183350-overview

  Whipple disease is extremely rare worldwide; only several hundred clinical cases have been reported, mostly from North America and western Europe. The incidence has been estimated to be less than 1 per 1,000,000. […] Whipple disease is most common in white males and rarely is described in females (male-to-female ratio: approximately 8-9:1). The route of disease transmission is not known but may be associated with occupational exposure to animals and soil. […] Whipple disease is usually observed in middle-aged and elderly persons (older than 40 y).

• #4 Whipple’s disease review, prevalence, mortality, and characteristics in the United States: A cross-sectional national inpatient study

  https://pmc.ncbi.nlm.nih.gov/articles/PMC9750640/

  Whipples disease admissions were significantly older than other hospitalizations, with a mean age of 60.21.6 years compared to 50.00.1. […] The prevalence of Whipples disease was 4.6 per 1 million hospitalizations during the study period. […] A disproportionate number of admissions occurred in the Midwest. […] High hospitalization rates in the Midwest support environmental and occupational disease transmission likely from the soil. […] We estimated the inpatient prevalence of WD to be 4.6 per 1 million hospitalizations, which is the first national estimate of WD prevalence in the inpatient setting. […] Our study is the first to examine geographic distribution of WD in the United States, and showed a greater risk of WD in the Midwest United States.

• #5 Orphanet: Whipple disease

  https://www.orpha.net/en/disease/detail/3452

  The annual incidence in Central European countries is estimated to be approximately 1/1,000,000. […] The disease may occur at any age with diagnosis most frequently made between 50 to 60 years of age. […] The circumstances facilitating infection and disease are unknown, but a genetic or acquired immunological predisposition is suspected. […] Lifelong clinical, non-invasive observation is advised in effectively treated patients, as late recurrences or de novo infections can occur.

• #6 Whipple’s Disease: Symptoms and Causes | Doctor

  https://patient.info/doctor/whipples-disease

  Whipple’s disease is an extremely rare condition. Incidence is estimated as between 1 to 6 cases per 1,000,000 people, per annum, globally. Its prevalence is estimated as 1.1 per million. […] Whipple’s disease is more common in: Middle-age and older individuals (mean age at diagnosis is 55 years). Men (85% of cases are in men). White people. Family clusters (suggesting an immunogenetic component). Certain geographic locations: most cases of Whipple’s disease are diagnosed in Western Europe and North America. […] HLA-B27 antigen; HLA-DRB1*13 and DQB1*06 alleles. Sewage plant workers, farmers and agricultural workers.

• #7 Whipple’s disease epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Whipple%27s_disease_epidemiology_and_demographics

  Whipple’s disease is a systemic disease among middle-aged white males in North America and western Europe. It affects males 8 times more than females. Few studies were done to evaluate the demographics of Whipple’s disease due to the sparsity of the disease. The incidence of Whipple’s disease is approximately 0.1 per 100,000 individuals and the prevalence is approximately 0.3 per 100,000 individuals in north-western Italy. […] The incidence of Whipple’s disease is approximately 0.1 per 100,000 individuals in north-western Italy. […] The incidence of Whipple’s disease is approximately 12 annually worldwide. […] Between 1907 and 1987, the incidence of Whipple’s disease was estimated to be 696 cases worldwide. […] The prevalence of Whipple’s disease is approximately 0.3 per 100,000 individuals in north-western Italy.

• #7 Whipple’s disease epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Whipple%27s_disease_epidemiology_and_demographics

  Whipple’s disease usually affects individuals of the caucasian race. Africans and Asians are less likely to develop Whipple’s disease. […] Males are more commonly affected by Whipple’s disease than females. The male to female ratio is approximately 8 to 1. […] The majority of Whipple’s disease cases are reported in North America and western Europe.

• #8 Whipple disease | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/whipple-disease?lang=us

  The incidence of Whipple disease is not known, one Swiss study estimated it at approximately 1 per 1.5 million per year. […] The peak age for presentation is in the fifth decade of life. Caucasians are most often affected, and men are affected eight times more commonly than women. […] The disease is more common in farmers and those who work with soil and livestock.

• #9

  https://link.springer.com/article/10.1007/s10620-018-5393-9

  Prior studies on the epidemiology of Whipples disease are limited by small sample size and case series design. We sought to characterize the epidemiology of Whipples disease in the USA utilizing a large population-based database. […] 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 Whipples disease, with an overall prevalence of 9.8 cases per 1 million. […] To our knowledge, this is the largest study to date examining the epidemiology of Whipples disease. In this large population-based study, the overall prevalence of Whipples 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.

• #10

  https://journals.lww.com/md-journal/fulltext/2022/12090/whipple_s_disease_review,_prevalence,_mortality,.13.aspx

  Whipples disease is a rare multiorgan systemic disease caused by Tropheryma whipplei infection that may present with a wide range of signs and symptoms. […] The prevalence of Whipples disease was 4.6 per 1 million hospitalizations during the study period. […] A disproportionate number of admissions occurred in the Midwest. […] High hospitalization rates in the Midwest support environmental and occupational disease transmission likely from the soil. […] We estimated the inpatient prevalence of WD to be 4.6 per 1 million hospitalizations, which is the first national estimate of WD prevalence in the inpatient setting. […] Our study is the first to examine geographic distribution of WD in the United States, and showed a greater risk of WD in the Midwest United States.

• #11 Whipple’s Disease | Abdominal Key

  https://abdominalkey.com/whipples-disease/

  Whipples disease is a rare disorder. The first comprehensive epidemiologic survey was performed by Dobbins in 1987, compiling information on 696 patients and including 617 published and 79 unpublished cases recorded through 1986. According to this analysis, Whipples disease is a sporadic disorder with a predilection for middle-aged white men. Data on age and sex were available for 664 patients; 86% were male, and the mean age at diagnosis was 49 years. Most patients were white; only 10 were African, one was a Native American, three patients were from India, and one was Japanese. Most of the patients originated from Europe (373 patients) or from the United States (246 patients). Within Europe, Germany (114 patients) and France (91 patients) were strongly represented. Relatively few cases originated from South America (11 patients) and Australia (13 patients).

• #12 Tropheryma whipplei Infections, Mexico, 2019–2021 – Volume 31, Number 5—May 2025 – Emerging Infectious Diseases journal – CDC

  https://wwwnc.cdc.gov/eid/article/31/5/24-1046

  Whipples disease is rarely diagnosed in Latin America. […] WD is a rare disease with a prevalence of 9.8 cases/1 million inhabitants in the United States and is most frequently identified in White men 50 years of age. […] Classic WD has been reported extensively in western Europe, Canada, and in the United States. The disease has been described only rarely in countries in Latin America, including a probable case in Mexico. […] Identification of T. whipplei has epidemiologic implications because of its potential for transmission between humans through saliva or feces. […] In conclusion, our findings highlight the need for more specific and widely available diagnostic tests for T. whipplei in Mexico and across Latin America, particularly molecular diagnostic assays, to enable characterization of T. whipplei in the region.

• #13 Whipple’s Disease | Abdominal Key

  https://abdominalkey.com/whipples-disease/

  A small epidemiologic study from western Switzerland calculated the incidence of Whipples disease to be approximately 0.4 per million of the population per year. A similar incidence of 0.4 per million per year was estimated for Germany. An epidemiologic analysis of 110 patients with Whipples disease in Germany, identified between 1965 and 1995, noted the incidence of cases to be relatively stable over three decades and a relatively even geographic distribution of the patients residences. There are only a few observations of geographically confined case clusters (up to seven cases). […] In recent decades, several studies have indicated a statistically significant increase in the age of patients at diagnosis and an increasing percentage of female patients. Currently, patients are first diagnosed at a mean age of 56 years. It has been speculated that the increasing use of antibiotics for unrelated complaints may be a contributing factor in delaying the age of onset of Whipples disease.

• #14 Whipple’s Disease | Musculoskeletal Key

  https://musculoskeletalkey.com/whipples-disease-2/

  Whipples disease is a rare condition, and little is known about its epidemiology. There is no universally accepted incidence rate, but there have been estimates of a worldwide annual incidence of 12 new cases per year. The incidence may be higher than previously thought owing to the establishment of PCR testing, which has definitively assisted in the diagnosis of this illness. […] Whipples disease tends to present in middle age and is more common in males. As there is often a delay in the diagnosis attributable to nonspecific symptoms at presentation as well as antibiotic use before the expression of the most classic symptoms of the disease, the true age of onset is likely younger. Whipples disease has been reported in patients from 3 months to 83 years old, and a clinical review of 52 cases by Durand and colleagues found that in such a patient population, the mean age of diagnosis was between 48 and 54 years old.

• #15 Whipple’s disease in the elderly | Gastroenterología y Hepatología

  https://www.elsevier.es/es-revista-gastroenterologia-hepatologia-14-articulo-whipple39s-disease-in-elderly-S0210570523004934?newsletter=true

  Whipple’s disease (WD) predominantly affects white males (7395%) with peak incidence rate between 40 and 50 years. […] The total age range was 2379 years with maximum incidence rate occurring between 40 and 69 years (68% of total cases). Therefore, the conclusion is that it affects middle-aged individuals. […] In other studies, an advanced age has been associated with higher risk of contracting WD. The prevalence was from 7.9 cases per 1 million individuals 65 years. […] A large scale epidemiological study associated an advanced age with higher risk for WD, where prevalence reached maximum of 39.2 cases per 1 million in age group of 8084. Authors concluded that prevalence of WD was more common in individuals over 65 years, which contrasted with previous studies.

• #16 Whipple’s disease: a case report and review of literature – MedCrave online

  https://medcraveonline.com/MOJCR/whipplersquos-disease-anbspcase-report-and-review-of-literature.html

  Whipple’s disease (WD) is a rare condition. Few epidemiologic studies showed its annual incidence is less than 1 per 1,000,000 per annum. It is has a specific predilection for middle aged white males, about 86% with male-to-female ratio: approximately 8-9:1. There is a slight increase in the rate of women being diagnosed as Whipple’s disease in recent years. It occurs predominantly in those of Caucasian ethnicity, suggesting a genetic predisposition in that population. The disease is mainly diagnosed in the middle age (median 49 years), however some studies showed that the age of diagnosis had shifted recently to older age. This could be attributed to the increasing use of antibiotics to unrelated conditions had led to a delay in the onset of WD and this also can also be seen in our patient.

• #17 Whipple’s disease – Wikipedia

  https://en.wikipedia.org/wiki/Whipple%27s_disease

  The disease is regarded as extremely rare, with an incidence (new number of cases per year) of one case per million people. The patients are predominantly male, with various studies and sources typically reporting a ratio between 2:1 and 3:1 of male to female patients. […] In the United States and some other countries, it occurs predominantly in those of Caucasian ethnicity, suggesting a possible genetic predisposition in that population. […] Several lines of evidence suggest that some defect inherited or acquired in immunity is required for it to become pathogenic. […] The disease is usually diagnosed in middle age (median 49 years). Studies from Germany have shown that age at diagnosis has been rising since the 1960s.

• #18 Whipple’s disease: the great masquerader—a high level of suspicion is the key to diagnosis | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-021-01664-1

  Whipples disease (WD) is an infectious disease caused by the gram-positive bacterium Tropheryma whipplei. There are few studies on the prevalence and incidence of the disease. In a recent study from Italy, the prevalence was estimated to be 3 per 1,000,000 people. Most cases are reported from North America and Europe. According to one study of 664 patients from the United States, 86% of cases occurred in men, 98% were of Caucasian origin, with an average age at diagnosis of 49 years. Most of the patients were farmers with occupational exposure to soil or animals. In a German study, the percentage of women was higher, 22%, with a median age of 51 at the time of diagnosis. […] The disease primarily affects the small intestine but several other organs may be involved, e.g. joints, brain, lungs, and heart. Whipples disease can be lethal if not treated. The natural course of classic WD is characterized by two stages. In a first, often long-lasting prodromal stage, three-quarters of patients experience non-specific symptoms, where arthritis is the first and most common manifestation. In a second stage, the symptomatology is dominated by gastrointestinal manifestations, such as weight loss, nausea, and diarrhea. WD is therefore difficult to diagnose since it often has atypical manifestations and lymphadenopathy that lead to suspicion of lymphoma.

• #19 Whipple disease: Symptoms, causes, and diagnosis

  https://www.medicalnewstoday.com/articles/188228

  Men are more susceptible than women, and 87 percent of people with Whipple disease are men aged between 40 and 60 years. […] In the United States, Whipple disease affects fewer than one in every million people each year. In developing countries, it is more prominent, especially among children who live in areas where the sanitation system is poor. […] In Senegal, in 2011, it was reported that nearly 44 percent of children aged between 2 and 10 years had the bacteria in their stools.

• #20 SciELO Brazil – Whipple’s disease: a fatal mimic Whipple’s disease: a fatal mimic

  https://www.scielo.br/j/acrep/a/rdgGFSd8mWMHffytDSBjFHK/

  Whipples disease (WD) is an exceedingly rare systemic infection caused by the bacterium Tropheryma whipplei (T. whipplei), a fastidious, bacilliform, phylogenetically gram-positive bacteria characteristically positive for periodic acid schiff histochemical stain and diastase resistant (PAS-D+).1 WD presents with non-specific gastrointestinal symptoms and malabsorption. The disease reportedly predominantly affects middle-aged/elderly men (the majority of reported cases are in men over 55 years old), which is purportedly due to its association with wastewater workers and farmers (who are predominantly male) and the smoldering disease course that can take many years to manifest and correctly diagnose.2-4 However, recent epidemiological studies have suggested that the contemporary prevalence of WD may be comparable in men and women in the US.5

• #21 Whipple’s Disease | Musculoskeletal Key

  https://musculoskeletalkey.com/whipples-disease-2/

  Whipples disease affects males more often than females in an 8:1 ratio. However, recent studies have noted an increase in female patients from 13% to 20% compared with earlier data. The gender predisposition of Whipples disease is largely unexplained. It may be that similarly to ankylosing spondylitis, the disease may be less symptomatic in women. Although there is no evidence supporting a familial prevalence of the disease, genetic causes of disease expression have been proposed. In particular, an X-linked pattern of inheritance of regulatory genes integral in the expression of cytokines has been suggested. In addition, one review noted that 26% of patients with Whipples disease were positive for HLA B27. […] Geographic and ethnic distributions also differ. In 696 cases studied, 55% were from Europe and 38% were from North America, with only a minority of cases in Hispanics, blacks, Indians, and Asians. Farmers and people working outdoors have been noted to be more likely to contract Whipples disease, suggesting that there may be a zoonotic component of transmission. This concept is particularly attractive, as T whipplei is gram-positive bacterium in the Actinomyces family, a group of bacteria active in decomposition of the soil and organic materials. Although many cases have been noted to occur in rural areas and the disease can occur in local clusters, no environmental factors or habitats have been definitively associated with the disease. The small intestines of a variety of domesticated animals have been tested for the presence of T whipplei. Although none of the samples were positive, the sample size was small and extraintestinal tissue was not sampled, so the presence of T whipplei in animals cannot be excluded. In a study that tested 38 samples of wastewater for DNA specific to T whipplei, 25 of the 38 samples tested positive, supporting the argument that T whipplei may be acquired through the environment.

• #22 Whipple disease diagnosed by enteroscopy: first case report in Colombia of an underdiagnosed disease and literature review | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-020-01302-2

  Whipples disease is a rare systemic disease caused by a gram-positive bacillus called Tropheryma whipplei. […] This is a rare disease, the annual incidence of this entity has been 12 cases per year worldwide. […] It is more common among farmers, sewer workers, people working with plants and soil or exposed to animal feces. […] In Colombia there are no cases reported. […] This is a rare entity and difficult to diagnose given the diversity of the clinical manifestations according to the organ affected. […] It is important to consider this entity in people with recent trips to endemic areas where polluted water acts as a possible source of infection. […] Other predisposing factors are immunosuppression and human immunodeficiency virus (HIV) positive patients. […] The diagnosis is usually made with endoscopic procedures and biopsies of the affected tissue.

• #23 Whipple’s Disease | Abdominal Key

  https://abdominalkey.com/whipples-disease/

  One remarkable epidemiologic feature in Dobbins analysis was the strong representation of patients with occupations in the farming and building trades, involving outdoor work or frequent contact with animals or soil: of 191 patients for whom data were available, 43 (22%) were farmers and 10 (5%) were carpenters; patients in all farming-related trades accounted for 34% of the total; by comparison, the fraction of farm workers among the total workforce in the analyzed countries was approximately 10%.

• #24 Whipple’s Disease | Concise Medical Knowledge

  https://www.lecturio.com/concepts/whipples-disease/

  Whipple’s disease is a rare disorder with worldwide distribution. Overall incidence is 13 in 1 million individuals. Increased prevalence is noted in farmers (since T. whipplei is soil-dwelling) and sewage workers. Most common in White males (male:female ratio = 8:1). Mean age at symptom onset: 49 years. […] Epidemiology, surveillance data indicate that Whipple’s disease is possibly associated with HLA-B27 haplotype.

• #25 Whipple Disease – Gastrointestinal Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/gastrointestinal-disorders/malabsorption-syndromes/whipple-disease

  In the United States, Whipple disease is rare, affecting White individuals over age 30 and more commonly over age 60; in some parts of the world, however, it is endemic (1, 2). […] Some, but not all, studies report a male predominance. […] Affected patients may have subtle defects of cell-mediated immunity that predispose to infection with T. whipplei (3, 4).

• #26 Whipple’s Disease: What Is It, Causes, Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/5958-whipples-disease

  While the bacterium that causes the disease is common, Whipples disease is very rare. Experts analyzing Whipples disease cases estimate it affects 9 in 1 million people in the U.S. Not everyone who encounters T.whipplei develops the disease. […] Experts know that a type of bacteria often found in dirt causes Whipples disease. Anyone who encounters the bacteria could be at risk of infection. But Whipples disease is rare, and its not clear why more people dont develop the disease after exposure. […] People with weak immune systems or who take immunosuppressants have an increased risk of developing the disease. If thats your situation, its a good idea to wear gloves or to wash your hands thoroughly any time you work in dirt and mud.

• #27 An unusual presentation of Whipples disease: adenopathies, polyarthralgia and dermatomyositis-like symptoms

  https://www.explorationpub.com/Journals/em/Article/1001113

  WD can affect almost any organ system and is accordingly classified between classic systemic disease and localized or extraintestinal disease. […] WD is frequently associated with rheumatic manifestations (60% of the cases), which precede the gastrointestinal signs in three fourths of the patients. […] The use of immunosuppressive drugs for articular treatment (the illness being sometimes misdiagnosed as seronegative rheumatic disease) may accelerate the onset of intestinal or other systemic illness. […] The most frequently used treatment included a 2-week intravenously cephalosporins followed by oral sulfonamides for 12 years. […] Without proper antibiotic treatment, the disease invariably culminates in dissemination and is potentially fatal. […] WD is a rare systemic infection with T. whipplei, which is still a challenge to diagnose as its clinical manifestations are not specific, resembling many other systemic diseases and may manifest late during the course of the disease.

• #28 Frontiers | Tumor Necrosis Factor Inhibitors Exacerbate Whipple’s Disease by Reprogramming Macrophage and Inducing Apoptosis

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.667357/full

  Several studies have reported that in the context of undiagnosed, subclinical WD, immunosuppression with corticoids or methotrexate and/or biological therapies such as anti-TNF drugs that are commonly given to treat unclear joint manifestations could accelerate and aggravate the onset of symptoms. […] In a systemic review from 19 publications, 41 patients have been diagnosed for WD while treated for unclear arthropathy with etanercept, infliximab, adalimumab alone or in combination. […] Overall, our study highlights the changes induced by TNFI in the context of T. whipplei infection. Such changes may constitute or at least contribute to the biological basis of the exacerbation of WD and suggest that in addition to M. tuberculosis and HBV, patients with unexplained arthropathy should be screened for T. whipplei infection prior to introduction of anti-TNF therapy.

• #29

  https://link.springer.com/article/10.1007/s15010-002-3083-0

  Little is known about the epidemiology of Tropheryma whipplei and its prevalence in people without clinical signs of Whipple’s disease. […] The prevalence of T. whipplei DNA in patients and in controls was 4.2% (95% CI 2.07.6% ) and 7.0% (95% CI 4.011.3%), respectively. […] We conclude that the asymptomatic carrier state of T. whipplei indeed exists and that it is much more frequent than the rare Whipple’s disease.

• #30 Whipple’s disease: the great masquerader—a high level of suspicion is the key to diagnosis | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-021-01664-1

  WD is probably an under-diagnosed disease, since the bacterium T. whipplei can be found in asymptomatic carriers, and PCR-testing can detect the 16S rRNA of T. whipplei in healthy subjects. The disease has a higher prevalence among middle-aged men of European origin. In one study of healthy blood donors, one of 174 blood samples (0.6%) had positive PCR for T. whipplei, and, in another study of 620 healthy individuals, PCR was positive in 0.6% of saliva samples and in 1.5% of stool samples. […] Since most carriers of T. whipplei do not develop WD it is important to first rule out more common diseases with similar symptoms. These include inflammatory bowel disease, chronic infectious enterocolitis, rheumatic diseases, lymphoma, HIV infection, and tuberculosis. […] The diagnosis of WD is therefore made histopathologically with the detection of PAS-positive inclusions in histiocytes/macrophages in the lamina propria from small intestinal mucosal biopsies, but also other tissues such as articular fluid, lymph nodes, CSF and, heart valves.

• #31 Whipple’s disease epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Whipple%27s_disease_epidemiology_and_demographics

  In Europe, the prevalence of chronic carrier of Tropheryma whipplei is 1,000-11,000 per 100,000 of general adult population. […] In Senegal, the prevalence of chronic carrier of Tropheryma whipplei is 75,000 per 100,000 of children under 4 years of age. […] In Laos, the prevalence of chronic carrier of Tropheryma whipplei is 48,000 per 100,000 individuals by using quantitative real-time PCR (qPCR) of the feces of children. […] In Ghana, the prevalence of chronic carrier of Tropheryma whipplei is 27,500 per 100,000 individuals by using quantitative real-time PCR (qPCR) of the feces of children. […] The case-fatality rate of Whipple’s disease is approximately 100%, if left untreated. […] The case-fatality rate of treated Whipple’s disease is unknown. […] Whipple’s disease commonly affects individuals between 40 to 60 years of age; the median age at diagnosis is 50 years.

• #32 WHIPPLE’S DISEASE | Harrison’s Manual of Medicine

  https://harrisons.unboundmedicine.com/harrisons/view/Harrisons-Manual-of-Medicine/623716/all/WHIPPLE%E2%80%99S_DISEASE?q=PNEUMONIA

  Whipples disease is a chronic multiorgan infection caused by Tropheryma whipplei, a weakly staining gram-positive bacillus. Humans are the only known host. Seroprevalence studies indicate that 50% of people in Western Europe and 75% of those in rural Senegal have been exposed to T. whipplei, and the disease prevalence is estimated at 13 cases per 1 million population. The route of transmission is unclear but probably involves fecaloral spread and possibly involves droplet and/or airborne transmission.

• #33 Whipple’s Disease: Symptoms, Diagnosis, Treatment & More

  https://www.healthline.com/health/whipples-disease

  Whipples disease can be life threatening, but the condition is relatively rare, affecting only 1-3 people per million around the world. […] It is slightly more common in the United States, affecting 9.8 out of every 1 million people based on data collected between 2012 and 2017. […] Whipples disease is more common in people who are white, non-Hispanic, or those over 65 years old. […] T. whipplei bacterium is known to live in soil, so the disease can be more common among farmers. […] However, there is also evidence of person-to-person transmission, as well as through waste water. […] The rate for Whipples disease also tends to be higher in places that lack fresh water and proper sanitation. […] Currently, theres no known way to prevent Whipples disease. […] Even so, very few people who contract the bacterium will develop the disease. […] Elchert JA, et al. (2019). Epidemiology of Whipples Disease in the USA between 2012 and 2017: A population-based national study.

• #34 Can Whipple’s Disease Be Transmitted by Gastroscopes? | Infection Control & Hospital Epidemiology | Cambridge Core

  https://www.cambridge.org/core/journals/infection-control-and-hospital-epidemiology/article/can-whipples-disease-be-transmitted-by-gastroscopes/D796D2CA953F975476F1F6A5115A9191

  To determine whether disinfection protocols currently used for gastroscopes are effective against cultures of Tropheryma whipplei. […] The bactericidal activity of a disinfectant is usually considered significant if it causes a 5 log10 or greater reduction in viable bacterial titers. Disinfecting gastroscopes with 2% glutaraldehyde or peracetic acids for 20 minutes may be insufficient to prevent transmission of T. whipplei on the instruments or stop false-positive results on polymerase chain reaction.

• #35 Whipple disease | MedLink Neurology

  https://www.medlink.com/articles/whipple-disease

  Whipple disease is a rare central nervous system disease. […] Whipple disease has been estimated to occur in 4.6 million hospitalizations with a mean age of 60.2 +/- 1.6 years with males representing two-thirds of admissions. Most cases occur in the mid-West of the United States. The presentation of Whipple disease was heterogeneous, especially in older males. It is speculated that the high hospitalization in the mid-West is related to environmental factors and occupational exposure from soil. […] A study of 35 million individuals in the United States suggested that the one-year prevalence of first diagnosis of Whipple disease was 1 of 1,000,000 patients; the prevalence of asymptomatic carriage is possibly quite high due to fecal oral transmission, as supported by an observation that 48% of the European population have antibodies to Whipple disease, suggestive of a prior exposure to infections in middle age which may take years to manifest. […] Epidemiology is poorly understood, and no current method of prevention is known.

• #36 Whipple’s disease: the great masquerader—a high level of suspicion is the key to diagnosis | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-021-01664-1

  WD has a dismal prognosis if left untreated. It is important to treat with antibiotics that penetrate the blood-brain barrier given that T. whipplei often is present in the CSF. WD without CNS involvement is initially treated with intravenous ceftriaxone 2 g once daily or meropenem (three doses of 1 g/day) for 2 weeks, followed by maintenance therapy with oral TMP-SMX 160 mg/800 mg twice a day for 1 year. […] After discontinuation of antibiotic therapy, there is a 9-15% risk of T. whipplei to persist latently in the body for many years, mainly in patients with CNS involvement. The average interval between the end of antibiotic treatment and an ensuing relapse is 4.2 years but may occur even after 30 years. Therefore, annual check-ups are recommended for at least some years after the end of treatment.

• #37 An unusual presentation of Whipples disease: adenopathies, polyarthralgia and dermatomyositis-like symptoms

  https://www.explorationpub.com/Journals/em/Article/1001113

  Several years usually elapse between symptom onset, characteristic clinical manifestations and the diagnosis. […] WD should be taken into account because of its wide variety of clinical manifestations and because of its potential fatal outcome in the absence of treatment. […] The risk of sequelae and mortality rate are high if its not diagnosed and treated in a timely manner. […] To avoid misdiagnosis, clinicians should be aware of this unusual presentation of WD, mimicking an autoimmune disease with dermatomyositis-like presentation that should be a part of the list of the differential diagnosis, as delay in diagnosis may be life-threatening.

• #38 Frontiers | Tumor Necrosis Factor Inhibitors Exacerbate Whipple’s Disease by Reprogramming Macrophage and Inducing Apoptosis

  https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2021.667357/full

  Whipple’s disease (WD) is a rare chronic and systemic disorder, caused by the bacterium Tropheryma whipplei and characterized by diarrhea, abdominal pain, and weight loss. […] Advances in epidemiology and molecular biology have revealed that, beside WD, T. whipplei infections cover several clinical entities including localized chronic infections without digestive involvement, acute infections and asymptomatic carriage. […] Although initially thought as a rare disease caused by a rare bacterium, some specific, not yet fully resolved host immune deficiencies explain the rarity of the disease in front of the ubiquity of the bacterium. […] WD predominantly occurs in white middle-aged men. […] As a result, the mean time from joint symptom onset to the diagnosis is 6.7 years and is influenced by immunosuppressive therapy, such as corticosteroids or tumor necrosis factor (TNF) inhibitors.

• #39 Subcutaneous Nodules Caused by Tropheryma whipplei Infection – Volume 28, Number 3—March 2022 – Emerging Infectious Diseases journal – CDC

  https://wwwnc.cdc.gov/eid/article/28/3/21-1989_article

  Whipple disease was reported in 1907 and is a chronic infectious disease caused by the bacterium Tropheryma whipplei. […] Because of the variety and confusion of symptoms, the average time to diagnosis is 6 years. […] Whipple disease is a rare infectious disease. Nearly 80% of patients had arthralgia and digestive disorders before they were given a diagnosis. […] Whipple disease has been rarely reported in China. The few reports involved the central nervous system and respiratory system.

• #40 Whipple’s disease – Knowledge and References – Taylor & Francis

  https://taylorandfrancis.com/knowledge/Medicine_and_healthcare/Gastroenterology/Whipple%27s_disease/

  Whipple’s disease is a rare systemic infection caused by the bacterium Tropheryma whipplei that affects the gastrointestinal system and may also have extraintestinal manifestations such as chest pain, congestive heart failure, pericarditis, lymphadenopathy, and skin hyperpigmentation. […] Whipple’s disease is a rare infectious disease caused by bacteria, Tropheryma whipplei. […] T. whipplei was first thought to be an uncommon bacterium that caused a rare disease. Since the strain has been cultured, the genome has been sequenced and diagnostic tools have improved and come into more widespread use, leading to increase of knowledge about the bacterium, its epidemiology and pathogenicity. […] Classic Whipple’s disease and localized infections remain rare and fatal clinical entities without antibiotic treatment, whereas T. whipplei is a more common bacterium and associated with a wider diversity of clinical presentation than initially described, including frequent asymptomatic carriage and acute infections (gastro-enteritis, pneumonia, and bacteremia). […] Immunological and genetic predispositions to the disease are suggested due to the high prevalence of carriage and acute infection compared to the rarity of Whipple’s disease in target populations, notably in Africa and Asia.

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