Whippleʼs Disease With Axial and Peripheral Joint Destruction

Scheib, Jeffrey S.; Quinet, Robert

doi:10.1097/00007611-199006000-00024

Cited by 28 publications

(19 citation statements)

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“…Vertebral involvement is rare. Sarcoiliitis and spondylitis also associated with Whipple's disease (25,164). So far, only one case of spondylodiscitis, initially detected by broad-spectrum PCR and sequence analysis from an open biopsy specimen and later confirmed by PAS staining (in an ileum biopsy specimen only) and PCR (in ileum, duodenum, sigmoid, and colon biopsy specimens) has been reported (5).…”

Section: Clinical Manifestationsmentioning

confidence: 99%

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Whipple's Disease and “Tropheryma whippelii”

2001

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SUMMARY Whipple's disease is a rare bacterial infection that may involve any organ system in the body. It occurs primarily in Caucasian males older than 40 years. The gastrointestinal tract is the most frequently involved organ, with manifestations such as abdominal pain, malabsorption syndrome with diarrhea, and weight loss. Other signs include low-grade fever, lymphadenopathy, skin hyperpigmentation, endocarditis, pleuritis, seronegative arthritis, uveitis, spondylodiscitis, and neurological manifestations, and these signs may occur in the absence of gastrointestinal manifestations. Due to the wide variability of manifestations, clinical diagnosis is very difficult and is often made only years or even decades after the initial symptoms have appeared. Trimethoprim-sulfamethoxazole for at least 1 year is usually considered adequate to eradicate the infection. The microbiological diagnosis of this insidious disease is rendered difficult by the virtual lack of culture and serodiagnostic methods. It is usually based on the demonstration of periodic acid-Schiff-positive particles in infected tissues and/or the presence of bacteria with an unusual trilaminar cell wall ultrastructure by electron microscopy. Recently, the Whipple bacteria have been characterized at the molecular level by amplification of their 16S rRNA gene(s). Phylogenetic analysis of these sequences revealed a new bacterial species related to the actinomycete branch which was named “Tropheryma whippelli.” Based on its unique 16S ribosomal DNA (rDNA) sequence, species-specific primers were selected for the detection of the organism in clinical specimens by PCR. This technique is currently used as one of the standard methods for establishing the diagnosis of Whipple's disease. Specific and broad-spectrum PCR amplifications mainly but not exclusively from extraintestinal specimens have significantly improved diagnosis, being more sensitive than histopathologic analysis. However, “T. whippelii” DNA has also been found in persons without clinical and histological evidence of Whipple's disease. It is unclear whether these patients are true asymptomatic carriers or whether differences in virulence exist among strains of “T. whippelii” that might account for the variable clinical manifestations. So far, six different “T. whippelii” subtypes have been found by analysis of their 16S-23S rDNA spacer region. Further studies of the pathogen “T. whippelii” as well as the host immune response are needed to fully understand this fascinating disease. The recent cultivation of the organisms is a promising major step in this direction.

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Section: Clinical Manifestationsmentioning

confidence: 99%

“…Chronic pain is uncommon. Joint deformity or destructive joint changes associated with Whipple's disease are very rare (9,71,164). For unknown reasons, joint pain often diminishes after intestinal symptoms develop.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

Whipple's Disease and “Tropheryma whippelii”

2001

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“…1). The small joints such as the metacarpophalangeal and interphalangeal joints are spared [7][8][9]. The destructive lesions indicate septic arthritis due to T. whipplei, which can be cultured from the involved joints (7).…”

Section: Discussionmentioning

confidence: 99%

Atypical focal forms of Whipple's disease seen by rheumatologists

2015

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“…I n an article published in 1990, 1 we described the radiographic features of an HLA-B7-positive white male patient with Whipple disease and erosive polyarthritis and what were felt to be the conspicuously unusual findings of bilateral sacroiliitis, pancarpal narrowing, and cervical apophyseal fusion. Whipple disease has previously been characterized as being associated with a nonerosive arthritis, 2-8 although individual reports have challenged this notion.…”

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confidence: 99%

“…Whipple disease has previously been characterized as being associated with a nonerosive arthritis, 2-8 although individual reports have challenged this notion. 1,5,[7][8][9][10][11][12][13][14] In our initial paper, we provided the first description of bilateral sacroiliitis, cervical apophyseal fusion, and pancarpal narrowing occurring in the same patient with Whipple disease. Shortly thereafter, another patient was diagnosed with Whipple disease who also demonstrated these findings, raising the specter of a previously unrecognized radiographic triad in Whipple disease with advanced arthritis.…”

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confidence: 99%

Whipple Disease Revisited

Scheib

2004

JCR: Journal of Clinical Rheumatology

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Whipple disease is a relatively rare systemic disorder with protean manifestations among which arthropathic involvement is a frequently attendant and early manifestation. We report the second of 2 consecutively diagnosed Whipple patients seen in an outpatient rheumatology practice who exhibited the triad of the singularly seldom reported radiographic findings of sacroiliitis, cervical apophyseal fusion, and pancarpal narrowing is reported. Both unexplained recurrent arthritis and these radiographic features should lead one to consider this treatable disease.

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Whippleʼs Disease With Axial and Peripheral Joint Destruction

Cited by 28 publications

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Whipple's Disease and “Tropheryma whippelii”

Whipple's Disease and “Tropheryma whippelii”

Atypical focal forms of Whipple's disease seen by rheumatologists

Whipple Disease Revisited

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