RESUMO
Whipple's disease is a rare systemic infection caused by a diastase-resistant and Gram-positive bacillus called Tropheryma whipplei. The diagnosis of the disease is difficult and often late due to the variety of the symptoms. The objective of this study was to report a rare case of Whipple's disease treated at the Internal Medicine service of a hospital in the Midwest of Santa Catarina, Brazil. The patient was invited for an interview to describe the history of symptoms and previous treatments. Additionally, test results and treatments performed during hospitalization were assessed through the medical records. The case report describes a 39-year-old white male who presented, at the time of diagnosis, severe diarrhea, hematochezia, weight loss, fever, and inguinal adenomegaly, in addition to preceding migratory polyarthritis. After investigation, the diagnosis of Whipple's disease was achieved in conjunction with antibiotic treatment, with improvement of clinical and laboratory symptoms after 15 days. This case highlights the broad array of symptoms and the non-specific features of the Whipple's disease. Establishing an early diagnosis is essential for the success of the treatment, avoiding a long-lasting escalation of symptoms, which lead to cachexia and a devastating loss of the patient's quality of life.
RESUMO
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.
Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Doença de Whipple/complicações , Doença de Whipple/diagnóstico , Doença de Whipple/tratamento farmacológico , Europa (Continente) , Tropheryma , Antibacterianos/uso terapêuticoRESUMO
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.
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.
Assuntos
Hipertensão Pulmonar , Doença de Whipple , Idoso , Antibacterianos/uso terapêutico , Europa (Continente) , Humanos , Hipertensão Pulmonar/diagnóstico , Hipertensão Pulmonar/etiologia , Masculino , Pessoa de Meia-Idade , Tropheryma , Doença de Whipple/complicações , Doença de Whipple/diagnóstico , Doença de Whipple/tratamento farmacológicoRESUMO
INTRODUCTION: Wipple disease (WD) is a rare chronic disease caused by the bacillus Tropheryma whipplei. Constitutive, rheumatologic, gastrointestinal, cardiac, cerebral, lymphatic, cutaneous, and ophthalmological signs are possible systemic symptoms. However, thrombotic manifestations are rarely described as "stroke-like syndrome" or arterial thrombosis. Diagnosis is based on clinical manifestations and pathological examination. Laboratory findings may include anemia, leukocytosis, and thrombocytosis. OBJECTIVE: We report a case of venous thrombosis as initial manifestation of WD. CASE REPORT: We describe the case of a 53-year-old male with iliofemoral vein thrombosis followed by intermittent diarrhea, loss of appetite, abdominal distension, and bloating. A mild malnutrition state with a weight loss of 13 kg, pallor (+/4 +), presence of lower-limb edema (+/4 +), and hypertympanic distended abdomen occurred. Laboratory tests on admission revealed anemia, positive inflammatory activity tests, and normal coagulation. Endoscopic examination showed villous edema with white dotted infiltrates in the second duodenal portion and intestinal lymphangiectasia in the terminal ileum. Pathological examination revealed numerous macrophages with positive periodic acid-Schiff inclusions. Venous Doppler ultrasound showed extensive deep thrombosis on the left lower limb and recanalization of the femoral vein in the right lower limb. The patient was treated with ceftriaxone and enoxaparin sodium, which led to an improvement of gastrointestinal and thrombosis symptoms. COMMENTS: Hypercoagulability, endothelial damage, vasculitis, and blood stasis are present in T. whipplei infection, which are associated with the activation of inflammatory mechanisms as well as procoagulant and thromboembolic events. WD should be part of the differential diagnosis of diseases that cause venous thrombosis of unknown origin.
RESUMO
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.
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Whipple's disease is a rare and multisystemic bacterial disease caused by the Gram-positive bacillus Tropheryma whipplei. Since it is a rare disease with a varied clinical presentation, its diagnosis is often delayed. The joint manifestations may precede the typical syndrome in decades, being a challenge for specialists and internists. We report a case of Whipple's disease in a man with polyarthralgia whose diagnosis was established after extensive clinical deterioration over five years.
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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).
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Carcinoma/diagnóstico , Tropheryma/isolamento & purificação , Doença de Whipple/diagnóstico , Antibacterianos/uso terapêutico , Ceftriaxona/uso terapêutico , Diagnóstico Diferencial , Doença de Whipple/tratamento farmacológicoRESUMO
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).
Assuntos
Carcinoma/diagnóstico , Tropheryma/isolamento & purificação , Doença de Whipple/diagnóstico , Antibacterianos/uso terapêutico , Ceftriaxona/uso terapêutico , Diagnóstico Diferencial , Feminino , Humanos , Pessoa de Meia-Idade , Doença de Whipple/tratamento farmacológicoRESUMO
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.
Assuntos
Humanos , Masculino , Adulto , Doença de Whipple/diagnóstico , Biópsia , Ceftriaxona/administração & dosagem , Colonoscopia , Doença de Whipple/tratamento farmacológico , Antibacterianos/administração & dosagemRESUMO
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.