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

Abstract

Whipple's disease is a rare multiorgan systemic disease caused by Tropheryma whipplei infection that may present with a wide range of signs and symptoms. This study aim to comprehensively review and determine the inpatient prevalence, mortality, risk factors, and reasons for hospitalization of patients with Whipple's disease. ICD-10 codes were used to identify admissions with Whipple's disease during the years 2016 to 2018. Characteristics of admissions with and without Whipple's disease were compared. The most common reasons for hospitalization were identified in admissions with Whipple's disease. The prevalence of Whipple's disease was 4.6 per 1 million hospitalizations during the study period. Whipple's disease admissions were significantly older than other hospitalizations, with a mean age of 60.2 ± 1.6 years compared to 50.0 ± 0.1. Males were more likely to have Whipple's disease and represented approximately two-thirds of hospitalizations. A disproportionate number of admissions occurred in the Midwest. Patients with Whipple's disease were most commonly admitted for gastrointestinal disease, followed by systemic infection, cardiovascular/circulatory disease, musculoskeletal disease, respiratory disease, and neurological disease. High mortality was seen in admissions for central nervous system (CNS) disease. Whipple's disease has heterogeneous presentations for inpatient admissions, and disproportionately affects older males. High hospitalization rates in the Midwest support environmental and occupational disease transmission likely from the soil. Hospitalists should be aware of the various acute, subacute, and chronic presentations of this disease, and that acute presentations may be more common in the inpatient setting.

Clinical and laboratory predictors and prevalence of immune reconstitution inflammatory syndrome in patients with Whipple's disease

“…16 Another American registry-based study found a prevalence of WD of 4.6 per 1 million hospitalizations. 17 A previous study by our group found a prevalence in northern Italy of three WD cases per 1 million inhabitants. 15 Although our sample of 45 patients with WD is certainly worthy of consideration from a clinical point of view, it is nevertheless undeniable that, from a statistical point of view, 45 patients are insufficient to perform a more extensive statistical analysis.…”

“…whipplei infection, including only 18 cases of frank WD, were collected over 20 years 16 . Another American registry‐based study found a prevalence of WD of 4.6 per 1 million hospitalizations 17 . A previous study by our group found a prevalence in northern Italy of three WD cases per 1 million inhabitants 15 .…”

“…WD is a rare disorder with an insidious onset, which is difficult to diagnose and treat 15–17 . It is an infectious disease that, especially in its initial phase, resembles many rheumatological conditions.…”

“…WD is a rare disorder with an insidious onset, which is difficult to diagnose and treat. [15][16][17] It is an infectious disease that, especially in its initial phase, resembles many rheumatological conditions. The vast majority of patients with WD in their prodromal phase are quite often misdiagnosed as having seronegative arthritis and treated with immunosuppressive therapy.…”

Prolonged arthralgias in Whipple’s disease – A common but often overlooked symptom of a rare disease

“…3 In 1952, it was first thought to be of infectious aetiology as a patient with Whipple’s disease responded to treatment with chloramphenicol, but only in the year 2000, the bacterium was grown and isolated from the heart valve of a patient with blood culture negative endocarditis. 1 –3 T. whipplei is present in stool samples of 1%–11% of healthy individuals and in 12%–26% of sewage plant workers; 2 nonetheless it is estimated that less than 0.01% of carriers will develop Whipple’s disease, 4 and that susceptibility depends on host genetic factors, specifically the HLA (human leucocyte antigen) alleles DRB1*13 and DQB1*06, the latter potentially correlated with the immune reconstitution inflammatory syndrome. 1,2,4 Four distinct presentations could be observed: classical Whipple’s disease, localised infection, acute infection and asymptomatic carriage.…”