Enfermedad de whipple e hipertensión pulmonar reversible / Whipple's disease and reversible pulmonary hypertension

Resumen La enfermedad de Whipple es una enfermedad multisistémica crónica, causada por la bacteria Tropherima whipplei. Se han descripto aproximadamente 1200 casos en la literatura. La incidencia mundial se estima en 9.8 casos por millón de personas. Los datos provenientes de Sudamérica y Europa muestran que afecta a varones de mediana edad. Se cree que los factores inmunológicos del huésped son los que influyen en el curso de la infección y no el genotipo del agente. Dado que las características clínicas no suelen ser específicas y el espectro de manifestaciones en órganos individuales puede ser subestimado, el diagnóstico sigue siendo muy difícil. Presentamos un caso confirmado por histopatología con compromiso multisistémico. Consideramos importante su difusión dados los escasos casos documentados en Sudamérica y la relevancia de tener presente la sospecha diagnóstica para el abordaje terapéutico precoz que mejora el pronóstico de esta rara enfermedad.

Abstract 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.

Tropheryma whipplei as the cause of acute pneumonia / 中华检验医学杂志

Tropheryma whipplei (TW) is an opportunistic pathogen that can cause Whipple′s disease (WD). Recently, the biomedical field is concerned about whether the bacteria cause pneumonia. When BALF was detected by PCR, the TW positive rate was about 6.1%. Etiology status is generally described as “associated” or “probable”. Immunocompromised status is the susceptible factor; Different species of the same genus have been reported. The current method may have missed detection. All relevant information needs further study. This article reviews the acute pneumonia caused by TW and the detection technology.

Doença de Whipple: rara causa de má absorção intestinal - relato de caso / Whipple's Disease: a rare cause of intestinal malabsorption - case report

A doença de Whipple é uma infecção sistêmica rara, causada pela bactéria gram-positiva Tropheryma whipplei, usualmente encontrada no solo. Acomete com maior frequência homens, entre 40 e 60 anos. Tem como característica ser uma doença com sintomas inespecíficos, o que dificulta seu diagnóstico. No presente estudo, relatamos o caso de um paciente do sexo masculino, 57 anos, que apresentava como sintomas principais diarreia e perda de peso significativa. Realizada investigação diagnóstica durante internação, onde a endoscopia digestiva alta confirmou o diagnóstico, com biópsia compatível com doença de Whipple. O paciente do presente estudo foi a óbito no décimo dia de internação. Entretanto, a doença quando diagnosticada precocemente apresenta bom prognóstico, sendo importante considerar doença de Whipple no diagnóstico diferencial de doenças disabsortivas. O tratamento é baseado em antibioticoterapia prolongada.

Whipple's disease is a rare systemic infection caused by gram-positive bacterium Tropheryma whipplei, usually found in soil. It affects more often men between 40 and 60 years. Its characteristic is a disease with specific symptoms, which makes diagnosis difficult. In this study, we report the case of a male patient, 57, who had as main symptoms diarrhea and significant weight loss. Held diagnostic investigation during hospitalization, where endoscopy confirmed the diagnosis with a biopsy compatible with Whipple's disease. The patient in this study died on the tenth day of hospitalization. However, the disease when diagnosed early has a good prognosis, it is important to consider Whipple's disease in the differential diagnosis of malabsorptive diseases. Treatment is based on prolonged antibiotic therapy.

Tumoural form of Whipple's disease simulating carcinomatosis

Whipple's disease is a rare disease caused by the actinomycete bacteria Tropheryma whipplei, which cause intestinal infection. The most common symptoms are chronic diarrhoea, weight loss, abdominal pain, arthritis and neurological abnormalities, which can be fatal. This paper reports a case of a 57-year-old Brazilian woman with diarrhoea, vomiting, abdominal pain, appetite loss, intermittent fever, malaise, weight loss and malnutrition. Migratory polyarthralgia and recurrent visual scotomas preceded the symptoms. The retroperitoneal pseudotumour formation finding was associated with prolonged wasting syndrome, which did not respond to usual therapies, thus leading to the investigation of carcinomatosis disease. After laparotomy, biopsy and histochemical study of the lesions with negative results for malignancy, we proceeded to the investigation of Whipple's disease, which was then confirmed. The patient improved clinically and started gaining weight after using ceftriaxone (IV).

Whipple's disease in an Afro-Caribbean national / Enfermedad de whipple en un nacional afrocaribeño

Whipple's disease is a rare multi-organ infectious disease caused by Tropheryma whipplei. It is fatal without treatment. We report on a 40-year old Afro-Jamaican man who presented with a six-month history of weight loss and diarrhoea. Investigations revealed iron deficiency anaemia and hypoalbuminaemia. Upper gastrointestinal endoscopy revealed white patchy lesions in the duodenum. The duodenal biopsy showed broadening and thickening of the villi by a dense infiltrate of foamy histiocytes within the lamina propria and focally extending into the attached submucosa. Periodic Acid-Schiff stains were positive. Electron microscopy was confirmatory and polymerase chain reaction testing conclusively identified the organisms as T whipplei. Antibiotic treatment resulted in resolution of symptoms. Although the diagnosis of Whipple's disease is difficult, increased awareness should lead to an increase in reported cases with the improvements in diagnostic capabilities.

La enfermedad deWhipple es una rara enfermedad infecciosa multiorgánica causada por el Tropheryma whipplei. Es fatal sin tratamiento. Reportamos un hombre afro-jamaicano de 40 años que se presentó con una historia de seis meses de pérdida de peso y diarrea. Las investigaciones revelaron hipoalbuminemia y anemia ferropénica. La endoscopia gastrointestinal superior reveló lesiones blancas irregulares en el duodeno. La biopsia duodenal mostró la ampliación y engrosamiento de las vellosidades por un denso infiltrado de histiocitos espumosos dentro de la lámina propia, que se extienden hasta la submucosa adjunta. Las tinciones con ácido peryódico de Schiff fueron positivas. La microscopia electrónica fue confirmatoria y la prueba de la reacción en cadena de la polimerasa, identificó los organismos como T whipplei de forma concluyente El tratamiento antibiótico trajo como resultado la resolución de los síntomas. Si bien el diagnóstico de la enfermedad de Whipple es difícil, un aumento de la conciencia debe conducir a un aumento en los reportes de casos divulgados que reflejan un mejoramiento en la capacidad para hacer el diagnóstico.

Whipple's disease: rare disorder and late diagnosis / Doença de Whipple: patologia rara e de diagnóstico tardio

Whipple's disease is a rare systemic infectious disorder caused by the bacterium Tropheryma whipplei. We report the case of a 61-year-old male patient who presented to emergency room complaining of asthenia, arthralgia, anorexia, articular complaints intermittent diarrhea, and a 10-kg weight loss in one year. Laboratory tests showed the following results: Hb = 7.5 g/dL, albumin = 2.5 mg/dL, weight = 50.3 kg (BMI 17.4 kg/m²). Upper gastrointestinal endoscopy revealed areas of focal enanthema in the duodenum. An endoscopic biopsy was suggestive of Whipple's disease. Diagnosis was confirmed based on a positive serum polymerase chain reaction. Treatment was initiated with intravenous ceftriaxone followed by oral trimethoprim-sulfamethoxazole. After one year of treatment, the patient was asymptomatic, with Hb = 13.5 g/dL, serum albumin = 5.3 mg/dL, and weight = 70 kg (BMI 24.2 kg/m²). Whipple's disease should be considered a differential diagnosis in patients with prolonged constitutional and/or gastrointestinal symptoms. Appropriate antibiotic treatment improves the quality of life of patients.

Doença de Whipple é uma rara infecção sistêmica causada pelo Tropheryma whipplei. Caracteriza-se por fase prolongada de sintomas inespecíficos, levando longo período até o seu diagnóstico. Sem tratamento, pode ser grave e fatal, mas com antibioticoterapia tem ótima resposta clínica e laboratorial. Relatamos o caso de paciente masculino, 61 anos, internado por astenia, anorexia, diarréia intermitente e perda de 10 kg em um ano. Apresentava-se com hemoglobina (Hb) 7,5 g/dL, albumina de 2,5 mg/dL, peso 50,3 kg (IMC 17,4). Endoscopia digestiva alta com áreas de enantema focal da mucosa duodenal e biópsia compatível com doença de Whipple. O diagnóstico foi confirmado com PCR sérica positiva, sendo instituído tratamento com ceftriaxone seguido de sulfametoxazol-trimetropim. Após um ano de tratamento, encontrava-se assintomático, com Hb 13,5 g/dL, albumina sérica de 5,3 mg/dL e peso de 70 kg. Doença de Whipple deve fazer parte da lista de diagnósticos diferenciais em pacientes com sintomas constitucionais e/ou com queixas gastrointestinais com evolução prolongada. O tratamento antibiótico pode curar a infecção, recuperando a qualidade de vida do paciente.

Detection of Tropheryma whipplei DNA in Joint Fluid of Patients with Arthritis by Polymerase Chain Reaction

BACKGROUND: Whipple's disease is a chronic systemic infection caused by Tropheryma whipplei which chiefly involves gastrointestinal tract and joints. Because arthritis is often the first sign of Whipple's disease, Whipple's disease may be misdiagnosed in the early stage of illness as non-infectious inflammatory arthritis. Rheumatoid arthritis may also be caused by infectious agents, including T. whipplei. To elucidate the relationship between T. whipplei infection and various arthritis, we examined T. whipplei DNA in Korean patients by polymerase chain reaction (PCR). MATERIALS AND METHODS: With two T. whipplei-specific primers dervied from the partial heat shock protein 65 gene sequence, PCR was performed on 56 joint fluid samples:33 samples from RA patients, 18 samples from OA patients, and 5 samples from patients with other unflammatory arthritis. In addition, the same method was applied to find out the DNA of T. whipplei in 8 normal adults' saliva and 22 patients' gastric juice. Due to the difficulty in obtaining the reference strain of T. whipplei, the PCR was performed without the positive control. RESULTS: The PCR method did not reveal T. whipplei in joint fluid from 56 patients with arthropathies, 8 samples from saliva, and 22 samples from gastric juice. CONCLUSION: Though technical errors can not be excluded as a cause of absence of a positive case, our results suggest that T. whipplei infection may not be a common cause of various arthritis in Korea; T. whipplei may be associated with inflammatory arthritis indirectly by immune-mediated mechanism rather than by direct joint invasion. The examination of specimens other than joint fluid could be helpful and the study of larger number of patient is highly desirable.

Detection of Tropheryma whipplei DNA in Joint Fluid of Patients with Arthritis by Polymerase Chain Reaction

BACKGROUND: Whipple's disease is a chronic systemic infection caused by Tropheryma whipplei which chiefly involves gastrointestinal tract and joints. Because arthritis is often the first sign of Whipple's disease, Whipple's disease may be misdiagnosed in the early stage of illness as non-infectious inflammatory arthritis. Rheumatoid arthritis may also be caused by infectious agents, including T. whipplei. To elucidate the relationship between T. whipplei infection and various arthritis, we examined T. whipplei DNA in Korean patients by polymerase chain reaction (PCR). MATERIALS AND METHODS: With two T. whipplei-specific primers dervied from the partial heat shock protein 65 gene sequence, PCR was performed on 56 joint fluid samples:33 samples from RA patients, 18 samples from OA patients, and 5 samples from patients with other unflammatory arthritis. In addition, the same method was applied to find out the DNA of T. whipplei in 8 normal adults' saliva and 22 patients' gastric juice. Due to the difficulty in obtaining the reference strain of T. whipplei, the PCR was performed without the positive control. RESULTS: The PCR method did not reveal T. whipplei in joint fluid from 56 patients with arthropathies, 8 samples from saliva, and 22 samples from gastric juice. CONCLUSION: Though technical errors can not be excluded as a cause of absence of a positive case, our results suggest that T. whipplei infection may not be a common cause of various arthritis in Korea; T. whipplei may be associated with inflammatory arthritis indirectly by immune-mediated mechanism rather than by direct joint invasion. The examination of specimens other than joint fluid could be helpful and the study of larger number of patient is highly desirable.