Whipple's disease: a fatal mimic

Whipple's Disease, a rare diagnosis caused by the slow-growing bacterium Tropheryma whipplei, most often presents with the classically described signs of malabsorption due to gastrointestinal colonization. However, it can also have signs and symptoms that clinically overlap with rheumatic diseases, potentially resulting in misdiagnosis. Furthermore, treatment with modern potent biologic immunosuppressive agents and classic disease modifying anti-rheumatic drugs (DMARDs) can lead to serious exacerbation of undiagnosed infections. We present the case of a middle-aged woman with long term complaints of arthalgias, who was diagnosed with seronegative rheumatoid arthritis and subsequently treated for almost 7 years with such immunosuppressive therapies. The patient's disease course included chronic diarrhea that abruptly intensified and culminated in fatal hypovolemic shock/sepsis. A diagnosis of WD was made by autopsy examination, wherein several organ systems were found to be heavily involved by Tropheryma whipplei organisms, and their identification was confirmed with histochemical and molecular evaluation. Notably, most bacterial organisms were located deeply in the submucosa/muscularis of affected organs, a practical reminder to practicing pathologists that challenges the classic histopathologic description of Whipple disease as an infiltration of predominantly lamina propria, and the potential for sampling bias in typically superficial endoscopic biopsies during routine procedures.

Enfermedad de whipple en paciente HIV positivo / Whipple disease in an HIV patient

La enfermedad de Whipple es una enfermedad crónica sistématica de infrecuente presentación, causada por una bacteria, Tropheryma Whipplei, del orden Actinomycetales. Existen cerca de 1.000 casos reportados en la actualidad. Presentamos un caso de enfermedad de Whipple en un paciente HIV (+).

Whipple's disease. Report of five cases with different clinical features

Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up

Enfermedad de Whipple: caso clínico y revisión bibliográfica / Whipple's disease: case report and bibliographic review

Se presenta un caso de enfermedad de Whipple confirmado, en una mujer de 61 años, por estudio histológico de biopsias escalonadas de duodeno y yeyuno.Se señala la etiología bacteriana y el carácter sistémico de la afección, que es producida por la Tropheryma whippelii, que es un bacilo Gram positivo con forma de hoz. Se describe el cuadro clínico que es esencialmente polimorfo pero dentro del cual destacan la diarrea crónica de tipo alto, con caracteres de síndrome de malabsorción y marcado compromiso progresivo del estado general; las artralgias y poliartritis y la fiebre. La enfermedad responde favorablemente a diversos antibióticos que deben administrarse en forma prolongada para intentar reducir las recurrencias que son muy frecuentes y que comprometen muchas veces al sistema nervioso central

Pancreaticogastrostomía en una pancreatoduodenectomía con preservación del piloro: reporte de un caso / Pancreaticogastrostomy in a pancreatoduodenectomy with prevention of pyloric: presentation a case

La alta morbimortalidad asociada a la Operación de Whipple estandar se ha reducido en los últimos años, sin embargo, aún observan un gran número de complicaciones relacionadas con el muñon pancreático, tales como fugas de la anastomosis pancreatoyeyunal y disfunción endo y exocrina; otra serie de complicaciones se ven en relación a la gastrectomía como son el dumping, diarrea, alteraciones en la absorción y la úlcera marginal. La pancreaticogastrostomía acompañada a una pancreatoduodenectomía con preservación del píloto se presenta como una combinación excelente para reducir tales complicaciones. Nosotros presentamos el caso de un paciente masculino de 57 años de edad al cual se le realizó el procedimiento; posterior a ello, se realizó una valoración endoscópica y radiológica encontrando una mucosa de características normales y una función pilórica y vaciamiento gástrico adecuado. El quimismo gástrico resultó hiper-secretante, posiblemente debido a la alcalinización del antro gástrico por la secreción pancreática. La evaluación endocrina del páncreas determinado por una curva de tolerancia glucosada fue normal al igual que la evaluación exocrina a través del Test de Pancreolauryl