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Clinical Microbiology Reviews Apr 2017Whipple's disease is a rare infectious disease that can be fatal if left untreated. The disease is caused by infection with , a bacterium that may be more common than... (Review)
Review
Whipple's disease is a rare infectious disease that can be fatal if left untreated. The disease is caused by infection with , a bacterium that may be more common than was initially assumed. Most patients present with nonspecific symptoms, and as routine cultivation of the bacterium is not feasible, it is difficult to diagnose this infection. On the other hand, due to the generic symptoms, infection with this bacterium is actually quite often in the differential diagnosis. The gold standard for diagnosis used to be periodic acid-Schiff (PAS) staining of duodenal biopsy specimens, but PAS staining has a poor specificity and sensitivity. The development of molecular techniques has resulted in more convenient methods for detecting infections, and this has greatly improved the diagnosis of this often missed infection. In addition, the molecular detection of has resulted in an increase in knowledge about its pathogenicity, and this review gives an overview of the new insights in epidemiology, pathogenesis, clinical manifestations, diagnosis, and treatment of infections.
Topics: Anti-Bacterial Agents; Humans; Tropheryma; Whipple Disease
PubMed: 28298472
DOI: 10.1128/CMR.00033-16 -
Postgraduate Medical Journal Dec 2000Whipple's disease is a systemic bacterial infection and the common though not invariable manifestations are diarrhoea, weight loss, abdominal pain, and arthralgia.... (Review)
Review
Whipple's disease is a systemic bacterial infection and the common though not invariable manifestations are diarrhoea, weight loss, abdominal pain, and arthralgia. Arthritis or arthralgia may be the only presenting symptom, predating other manifestations by years. Virtually all organs in the body may be affected, with protean clinical manifestations. Various immunological abnormalities, some of which may be epiphenomena, are described. The causative organism is Tropheryma whippelii. The disease is uncommon though lethal if not treated. Recent data suggest the disease occurs in an older age group than previously described. The characteristic histopathological features are found most often in the small intestine. These are variable villous atrophy and distension of the normal villous architecture by an infiltrate of foamy macrophages with a coarsely granular cytoplasm, which stain a brilliant magenta colour with PAS. These pathognomonic PAS positive macrophages may also be present in the peripheral and mesenteric lymph nodes and various other organs. The histological differential diagnoses include histoplasmosis and Mycobacterium avium-intercellulare complex. The clinical diagnosis of Whipple's disease may be elusive, especially if gastrointestinal symptoms are not present. A unique sign of CNS involvement, if present, is oculofacial-skeletal myorhythmia or oculomasticatory myorhythmia, both diagnostic of Whipple's disease. A small bowel biopsy is often diagnostic, though in about 30% of patients no abnormality is present. In patients with only CNS involvement, a stereotactic brain biopsy can be done under local anaesthetic. A recent important diagnostic test is polymerase chain reaction of the 16S ribosomal RNA of Tropheryma whippelii. Whipple's disease is potentially fatal but responds dramatically to antibiotic treatment. In this review the current recommended treatments are presented. The response to treatment should be monitored closely, as relapses are common. CNS involvement requires more vigorous treatment because there is a high rate of recurrence after apparently successful treatment.
Topics: Anti-Bacterial Agents; Brain Diseases; Gastrointestinal Diseases; Humans; Whipple Disease
PubMed: 11085766
DOI: 10.1136/pmj.76.902.760 -
Medicine and Pharmacy Reports Aug 2021Whipple's disease (WD) is a rare chronic systemic disease caused by the Gram-positive bacillus Tropheryma whipplei. Despite over 100 years of observation and study...
BACKGROUND
Whipple's disease (WD) is a rare chronic systemic disease caused by the Gram-positive bacillus Tropheryma whipplei. Despite over 100 years of observation and study history of this disease, it still remains a difficult diagnostic and therapeutic challenge.
CLINICAL CASE PRESENTATION
Authors report the case of a 38-year-old man with long-time PPIs treatment because of GERD and no other clinical and paraclinical symptoms. Endoscopic slightly enlarged villi, accumulation of whitish matter at the tip of the villi of distal duodenal mucosa and periodic acid-Schiff staining positive inclusions in the stromal tissue may be typical signs of Whipple's disease. In discussion the possible explication of this case are presented.
CONCLUSIONS
It is still a challenge to diagnose Whipple's disease. Histological findings may confirm the diagnosis in patients with a combination of typical clinical manifestations, but histological signs alone are not pathognomic, and are not enough for a definitive diagnosis.
PubMed: 34527918
DOI: 10.15386/mpr-2237 -
Cureus Jul 2023Whipple's disease is a rare systemic disease caused by a infection. Although older literature reports a low rate of incidence, case reports continue to rise due to...
Whipple's disease is a rare systemic disease caused by a infection. Although older literature reports a low rate of incidence, case reports continue to rise due to increased awareness of the disease. Classic Whipple's disease presents as weight loss, diarrhea, and arthralgia and may involve the heart, central nervous system (CNS), or any other organ system. Some patients with Whipple's disease do not have the classic signs and symptoms of the disease. We present a case of Whipple's disease in a patient with poor appetite, weight loss, and granulomatous inflammation of various organs, including the kidneys and spleen, mimicking sarcoidosis. She had presented three years earlier with acute kidney injury (AKI) and hypercalcemia. The renal biopsy revealed diffuse granulomatous interstitial nephritis. Both AKI and hypercalcemia resolved with prednisone; however, her weight loss and decreased appetite continued. The initial positron emission tomography (PET) scan showed increased fluorodeoxyglucose (FDG) avidity in the spleen and large intestine, and the splenic biopsy revealed non-caseating granulomas. A diagnosis of sarcoidosis was made, and she was started on methotrexate with prednisone. Nevertheless, the weight loss and poor appetite were relentless. A repeat PET scan showed increased FDG avidity in loops of the small and large intestines. A small intestinal biopsy revealed positive periodic acid-Schiff (PAS) and negative acid-fast bacilli (AFB) revealing the diagnosis of Whipple's disease. Whipple's disease should be considered in the differential diagnosis of sarcoidosis, especially in those patients worsening on standard immunosuppression.
PubMed: 37575808
DOI: 10.7759/cureus.41839 -
Surgical Neurology International 2022Whipple disease (WD) is an infection caused by , which might present in three different forms: classical, localized, and isolated in the central nervous system (CNS). (Review)
Review
BACKGROUND
Whipple disease (WD) is an infection caused by , which might present in three different forms: classical, localized, and isolated in the central nervous system (CNS).
METHODS
We report the result of a systematic review of the literature on WD unusually presenting with exclusively neurological symptoms, including two previously unpublished cases. A description of two cases with isolated CNS WD was performed, as well as a literature search in , and .
RESULTS
Two male adult patients presented with exclusively neurological symptomatology. Both magnetic resonance imaging (MRI) showed an intracranial mass suggestive of brain tumor. The histopathological examination was consistent with WD, with no systemic involvement. In the review of the literature, 35 cases of isolated CNS WD were retrieved. The median age at diagnosis was 43.5 (IQR 31.5-51.5). In 13 patients, the MRI showed a brain mass consistent with a brain tumor. The most common finding in the biopsy was the periodic-acid Schiff-stained foamy macrophages. Only five cases presented the pathognomonic sign of oculomasticatory myorhythmia. Thirteen cases had an adverse outcome that resulted in death during follow-up, whereas another 13 improved. The other nine patients remained stable or presented moderate improvement.
CONCLUSION
Isolated CNS WD is a rare disease that should be considered among the differential diagnosis of CNS mass lesions. Brain biopsy is necessary to establish the diagnosis. It is stressed in the literature that an extended antibiotic course is required to prevent relapses and to control the disease.
PubMed: 36324907
DOI: 10.25259/SNI_591_2022 -
Medicina 2021Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The...
Whipple's disease is a chronic mutisystem disease caused by the bacteria Tropherima whipplei. Approximately 1200 cases have been described in the literature. The worldwide incidence is estimated at 9.8 cases per million people. Data from South America and Europe show that it affects middle-aged males. It is believed that host immunological factors rather than agent genotypic traits influence the course of the infection. Since the clinical characteristics are usually nonspecific and the wide spectrum of manifestations in individual organs may be underestimated, the diagnosis remains challenging. We present a case with multisystem compromise confirmed by histopathology. We consider its publication important given the few cases documented in South America and the relevance of bearing in mind the importance of an early diagnosis for a prompt treatment that improves the prognosis of this rare disease.
Topics: Aged; Anti-Bacterial Agents; Europe; Humans; Hypertension, Pulmonary; Male; Middle Aged; Tropheryma; Whipple Disease
PubMed: 33611249
DOI: No ID Found -
Clinical Liver Disease Mar 2021
Review
PubMed: 33868662
DOI: 10.1002/cld.1004 -
Clinical and Diagnostic Laboratory... Jan 2001