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Cureus Jun 2023Whipple's disease (WD) is caused by , frequently found in lamina propria's macrophages in the small intestine. It is a rare and chronic systemic infection, and the...
Whipple's disease (WD) is caused by , frequently found in lamina propria's macrophages in the small intestine. It is a rare and chronic systemic infection, and the principal clinical manifestations are diarrhea, weight loss, abdominal pain, and arthralgia. The diagnosis is difficult mainly because of its rarity and should be considered in patients with arthralgias, diarrhea, abdominal pain, and weight loss after more common conditions have been excluded. The laboratory diagnosis is established by a duodenal biopsy. The treatment involves 14 days of intravenous antibiotics with good penetration in the cerebrospinal fluid (i.e., ceftriaxone) and one-year treatment with oral co-trimoxazole. Early diagnosis and proper treatment are crucial because it improves the prognosis. We report the case of a 58-year-old female with skin hyperpigmentation, loss of appetite and weight (16% of body weight in three months), nausea, upper abdominal pain, and diarrhea. Esophagogastroduodenoscopy and colonoscopy were performed to obtain biopsy samples, which, together with laboratory tests and microbiological studies, led to a diagnosis of Whipple's disease.
PubMed: 37415994
DOI: 10.7759/cureus.39963 -
IDCases 2021Whipple's disease (WD), caused by infection with the organism , is a rare disease that classically presents with diarrhea, weight loss, and polyarthralgia. Less...
Whipple's disease (WD), caused by infection with the organism , is a rare disease that classically presents with diarrhea, weight loss, and polyarthralgia. Less commonly, Whipple's Disease can presentation with endocarditis or neurologic infections. The authors report a patient with Whipple's Disease endocarditis whose initial presentation was acute lower extremity arterial occlusion, and review current literature regarding the epidemiology, diagnosis, treatment, and prognosis of Whipple's Disease endocarditis.
PubMed: 33868927
DOI: 10.1016/j.idcr.2021.e01105 -
Reumatologia 2021Whipple's disease is a rare, chronic, systemic disorder caused by infection. The most common symptoms are weight loss, arthralgia, diarrhea and abdominal pain. Other... (Review)
Review
Whipple's disease is a rare, chronic, systemic disorder caused by infection. The most common symptoms are weight loss, arthralgia, diarrhea and abdominal pain. Other organ involvement can also occur in the patients. Joint manifestations may mimic rheumatoid arthritis or spondyloarthritis. Arthalgia, arthritis, spondylodiscitis, bursitis and/or tenosynovitis are seen in the majority of the patients. This explains why some of the symptoms are misdiagnosed as those of rheumatic diseases. Understanding of Whipple's disease is important for differential diagnostics of several rheumatic symptoms.
PubMed: 33976464
DOI: 10.5114/reum.2021.105418 -
Pathologie (Heidelberg, Germany) Mar 2023We report on a 69-year-old man suffering from chronic progressive oligoarthritis (localized in metacarpal and knee joints), which clinically was interpreted as...
We report on a 69-year-old man suffering from chronic progressive oligoarthritis (localized in metacarpal and knee joints), which clinically was interpreted as steroid-sensitive seronegative chronic arthritis. The patient died from sudden death at the emergency department after a 4-week history of increasing cough and dyspnea (meanwhile obtaining negative testing results for SARS-CoV-2). During the autopsy, we found massive pancarditis affecting all cardiac compartments, in particular exhibiting constrictive pericarditis, myocarditis, and multivalvular endocarditis. Microscopically, interstitial myocarditis could be observed. Performing extensive molecular analyses, we detected Tropheryma whipplei in the tissue specimens of the heart, but not in various duodenal tissue probes or in the synovial membrane. Taken together, in the present case the cause of death was acute cardiac failure due to multivalvular pancarditis due to T. whipplei. Besides from classical symptoms and morphological signs, Whipple's disease may present with various features. Regarding the differential diagnosis of a chronic multisystem disorder with aspects of hitherto unknown arthralgia, Whipple's disease should be considered.
Topics: Male; Humans; Aged; Anti-Bacterial Agents; Myocarditis; Whipple Disease; Autopsy; COVID-19; SARS-CoV-2; Arthritis
PubMed: 36592174
DOI: 10.1007/s00292-022-01170-5 -
Tidsskrift For Den Norske Laegeforening... Jun 2008Whipple's disease is a rare infectious disease. This review describes the clinical picture, diagnostic aspects and treatment options. (Review)
Review
BACKGROUND
Whipple's disease is a rare infectious disease. This review describes the clinical picture, diagnostic aspects and treatment options.
MATERIAL AND METHODS
Based on a clinical case, a literature study was performed by searching PubMed (unlimited) with the words "whipples disease" combined with " TROPHERYMA WHIPPELII: " (118 hits) and " TROPHERYMA WHIPPLEI:" (65 hits). Titles and abstracts were used to identify other relevant articles.
RESULTS AND INTERPRETATION
Whipple's disease may affect several organ systems and thus causes a wide range of symptoms. The most common ones are weight loss, abdominal pain, diarrhoea and arthralgia. The diagnosis is based on PAS-POSITIVE MATERIAL: in duodenal biopsies and identification of gene fragments from the bacterium TROPHERYMA WHIPPLEI:. Presence of the bacterium may be confirmed by sensitive polymerase chain reaction assays. Left without treatment the disease may be lethal. Proper antibiotic treatment is imperative to heal the disease.
Topics: Actinobacteria; Anti-Bacterial Agents; Diagnosis, Differential; Female; Humans; Intestine, Small; Middle Aged; Polymerase Chain Reaction; Ultrasonography; Whipple Disease
PubMed: 18552903
DOI: No ID Found -
Alimentary Pharmacology & Therapeutics Apr 2015The classical form of Whipple's disease (WD), clinically characterised by arthropathy, diarrhoea and weight loss, is rare. Recently, other more frequent forms of... (Review)
Review
BACKGROUND
The classical form of Whipple's disease (WD), clinically characterised by arthropathy, diarrhoea and weight loss, is rare. Recently, other more frequent forms of Tropheryma whipplei infection have been recognised. The clinical spectrum includes an acute, self-limiting disease in children, localised forms affecting cardiac valves or the central nervous system without intestinal symptoms, and asymptomatic carriage of T. whipplei which is found in around 4% of Europeans. Genomic analysis has shown that T. whipplei represents a host-dependent or opportunistic bacterium. It has been reported that the clinical course of T. whipplei infection may be influenced by medical immunosuppression.
AIM
To identify associations between immunomodulatory treatment and the clinical course of T. whipplei infection.
METHODS
A PubMed literature search was performed and 19 studies reporting on immunosuppression, particularly therapy with tumour necrosis factor inhibitors (TNFI) prior to the diagnosis in 41 patients with Whipple?s disease, were evaluated.
RESULTS
As arthritis may precede the diagnosis of WD by many years, a relevant percentage (up to 50% in some reports) of patients are treated with immunomodulatory drugs or with TNFI. Many publications report on a complicated Whipple?s disease course or T. whipplei endocarditis following medical immunosuppression, particularly after TNFI. Standard diagnostic tests such as periodic acid-Schiff stain used to diagnose Whipple?s disease often fail in patients who are pre-treated by TNFI.
CONCLUSIONS
In cases of doubt, Whipple?s disease should be excluded before therapy with TNFI. The fact that immunosuppressive therapy contributes to the progression of T. whipplei infection expands our pathogenetic view of this clinical entity.
Topics: Anti-Bacterial Agents; Arthritis; Diagnosis, Differential; Humans; Immunoglobulins; Immunosuppressive Agents; Lymphocyte Count; Tumor Necrosis Factor-alpha; Whipple Disease
PubMed: 25693648
DOI: 10.1111/apt.13140 -
World Journal of Gastroenterology May 2009Whipple's disease was initially described in 1907. Over the next century, the clinical and pathological features of this disorder have been better appreciated. Most...
Whipple's disease was initially described in 1907. Over the next century, the clinical and pathological features of this disorder have been better appreciated. Most often, weight loss, diarrhea, abdominal and joint pain occur. Occasionally, other sites of involvement have been documented, including isolated neurological disease, changes in the eyes and culture-negative endocarditis. In the past decade, the responsible organism Tropheryma whipplei has been cultivated, its genome sequenced and its antibiotic susceptibility defined. Although rare, it is a systemic infection that may mimic a wide spectrum of clinical disorders and may have a fatal outcome. If recognized, prolonged antibiotic therapy may be a very successful form of treatment.
Topics: Anti-Bacterial Agents; Biopsy; Diagnosis, Differential; Duodenum; Humans; Nervous System Diseases; Tropheryma; Whipple Disease
PubMed: 19418579
DOI: 10.3748/wjg.15.2078 -
Frontiers in Immunology 2021is the agent of Whipple's disease, a rare systemic disease characterized by macrophage infiltration of the intestinal mucosa. The disease first manifests as arthralgia...
is the agent of Whipple's disease, a rare systemic disease characterized by macrophage infiltration of the intestinal mucosa. The disease first manifests as arthralgia and/or arthropathy that usually precede the diagnosis by years, and which may push clinicians to prescribe Tumor necrosis factor inhibitors (TNFI) to treat unexplained arthralgia. However, such therapies have been associated with exacerbation of subclinical undiagnosed Whipple's disease. The objective of this study was to delineate the biological basis of disease exacerbation. We found that etanercept, adalimumab or certolizumab treatment of monocyte-derived macrophages from healthy subjects significantly increased bacterial replication without affecting uptake. Interestingly, this effect was associated with macrophage repolarization and increased rate of apoptosis. Further analysis revealed that in patients for whom Whipple's disease diagnosis was made while under TNFI therapy, apoptosis was increased in duodenal tissue specimens as compared with control Whipple's disease patients who never received TNFI prior diagnosis. In addition, IFN-γ expression was increased in duodenal biopsy specimen and circulating levels of IFN-γ were higher in patients for whom Whipple's disease diagnosis was made while under TNFI therapy. Taken together, our findings establish that TNFI aggravate/exacerbate latent or subclinical undiagnosed Whipple's disease by promoting a strong inflammatory response and apoptosis and confirm that patients may be screened for prior to introduction of TNFI therapy.
Topics: Adult; Aged; Aged, 80 and over; Apoptosis; Female; Humans; Intestinal Mucosa; Macrophages; Male; Middle Aged; Tropheryma; Tumor Necrosis Factor Inhibitors; Whipple Disease
PubMed: 34093562
DOI: 10.3389/fimmu.2021.667357 -
Clinical Infectious Diseases : An... Feb 2001Whipple's disease was described in 1907 and was designated "intestinal lipodystrophy," despite the detection of bacteria in 1 specimen. This finding was later... (Review)
Review
Whipple's disease was described in 1907 and was designated "intestinal lipodystrophy," despite the detection of bacteria in 1 specimen. This finding was later substantiated by the success of antibiotic therapy, which resulted in dramatic clinical responses, and by use of electron microscopy, which detected monomorphic bacilli in affected tissues. Many attempts at culture failed, and these bacteria were characterized as actinomycetes for the first time by means of broad-range 16S rDNA amplification and molecular phylogenetic methods. The name "Tropheryma whippelii" was proposed for this bacterium. Whipple's disease is a systemic disease that affects many organ systems, producing protean manifestations. This article summarizes recent developments with regard to this topic as well as unanswered questions regarding the pathogenesis and acquisition of infection, the biology and ecology of the organism, the clinical spectrum of disease, diagnosis of the disease, and therapy.
Topics: Actinobacteria; DNA, Ribosomal; Humans; Microscopy, Electron; Polymerase Chain Reaction; RNA, Ribosomal, 16S; Whipple Disease
PubMed: 11170954
DOI: 10.1086/318512 -
Medical Principles and Practice :... 2020Whipple's disease is a very rare systemic infectious disease with an annual incidence of 3 in one million, which may be fatal if not diagnosed and treated appropriately.
OBJECTIVE
Whipple's disease is a very rare systemic infectious disease with an annual incidence of 3 in one million, which may be fatal if not diagnosed and treated appropriately.
CLINICAL PRESENTATION AND INTERVENTION
Herein we describe a 49-year-old patient admitted to the hospital with symptoms of severe malabsorption and diagnosed with Whipple's disease. The diagnosis was based on the histopathological findings of small intestine biopsies and PCR analysis.
CONCLUSION
Whipple's disease should be kept in mind while dealing with patients with severe malabsorption, even in the absence of accompanying features of the disease.
Topics: Diarrhea; Humans; Malabsorption Syndromes; Male; Middle Aged; Whipple Disease
PubMed: 30763942
DOI: 10.1159/000498909