RESUMO
Despite their rarity, Lyme disease and Whipple's disease are of significant importance in rheumatology, as both can manifest as chronic arthritis, presenting challenges in the differential diagnosis of inflammatory arthropathies. In Lyme disease, arthritis typically emerges as a late manifestation, usually occurring six months after the onset of erythema migrans. The predominant presentation involves mono- or oligoarthritis of large joints, with a chronic or remitting-recurrent course. Even with appropriate antimicrobial treatment, arthritis may persist due to inadequate immunological control triggered by the disease. In contrast, Whipple's disease may present with a migratory and intermittent seronegative poly- or oligoarthritis of large joints, preceding classic gastrointestinal symptoms by several years. Both disorders, particularly Whipple's disease, can be misdiagnosed as more common autoimmune rheumatic conditions such as rheumatoid arthritis and spondyloarthritis. Epidemiology is crucial in suspecting and diagnosing Lyme disease, as the condition is transmitted by ticks prevalent in specific areas of the United States, Europe, and Asia. On the contrary, the causative agent of Whipple's disease is widespread in the environment, yet invasive disease is rare and likely dependent on host genetic factors. In addition to erythema migrans in Lyme disease and gastrointestinal manifestations in Whipple's disease, neurological and cardiac involvement can further complicate the course of both. This article offers a comprehensive review of the epidemiological, pathophysiological, clinical, and therapeutic aspects of both diseases.
Assuntos
Artrite Reumatoide , Doença de Lyme , Doença de Whipple , Humanos , Reumatologistas , Doença de Whipple/diagnóstico , Doença de Whipple/tratamento farmacológico , Doença de Lyme/diagnóstico , Doença de Lyme/tratamento farmacológico , Doença de Lyme/epidemiologia , EritemaRESUMO
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
BACKGROUND: Whipple's disease is a rare systemic disease caused by a gram-positive bacillus called Tropheryma whipplei. First described in 1907 as an intestinal lipodystrophy with histological finding of vacuoles in the macrophages of the intestinal mucous. Usually the symptoms are localized according to the compromised organ. The differential diagnosis is wide. It can be fatal without proper treatment. Recurrence can occur in up to 33% of the cases and usually compromises the neurological system. CASE PRESENTATION: This article reports the case of a 46-year-old female patient with a history of a 6-month hypochromic microcytic anemia of unknown cause. She consulted for a 6-months oppressive abdominal pain located in the mesogastrium as well as abdominal distention associated with nausea and liquid stools; in addition, she had an 8-month small and medium joint pain, without edema or erythema. Physical examination without relevant findings. Multiple esophagogastroduodenoscopies with normal gastric and duodenal biopsies findings and a normal colonoscopy were performed. Endoscope capsule showed red spots in the duodenum and ulcerations in the jejunum and proximal ileum covered by fibrin; histological report showed macrophages with positive periodic acid-schiff reaction staining (PAS staining), disgnosing Whipple's disease. Antibiotics were initiated. The patient is currently in the second phase of treatment without gastrointestinal and joint symptoms. CONCLUSION: This is the first case reported in Colombia. It is a rare entity and difficult to diagnose reason why it is important to continue with clinical investigations to give more clarity about the onset and appropriate diagnose to avoid the delay in treatment of this entity.
Assuntos
Doença de Whipple , Antibacterianos/uso terapêutico , Colômbia , Endoscopia Gastrointestinal , Feminino , Humanos , Pessoa de Meia-Idade , Tropheryma , Doença de Whipple/diagnóstico , Doença de Whipple/tratamento farmacológicoRESUMO
Descrevemos um caso de doença de Whipple diagnosticada em paciente com síndrome diarreica crônica, emagrecimento intenso, pesquisa de DNA da Tropheryma whipplei positiva em fluido biológico e resposta excelente ao tratamento antibiótico.
A case of Whipple's disease diagnosed in a patient with chronic diarrhea syndrome, intense weight loss, positive DNA searching for Tropheryma whipplei, and excellent clinical response to antibiotic therapy is described.
Assuntos
Humanos , Masculino , Doença de Whipple/diagnóstico , Doença de Whipple/tratamento farmacológico , Combinação Trimetoprima e Sulfametoxazol/uso terapêuticoRESUMO
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
BACKGROUND: Whipple's disease is a rare and chronic bacterial disease with multiorganic involvement. Although there is no valid estimate of its actual prevalence, only about 1,000 cases have been reported. OBJECTIVE: To describe the demographic, clinical, laboratory, endoscopic and pathologic features, type of treatment used, its duration and response. METHODS: Based on the duodenal histology, we identified twelve cases of Whipple's disease in the Hospital Centre of Vila Nova de Gaia, between 1997 and 2010. RESULTS. Nine patients (75%) were male and the mean age at diagnosis was 58 years. All patients experienced at least one gastrointestinal and general symptom. Arthralgia were reported in four patients (33%) and occurred on average six years before the onset of gastrointestinal and general symptoms. In 10 patients the initial treatment was trimethoprim-sulfamethoxazole. The initial treatment duration was one year in 8 cases (80%). Seven patients (70%) had a symptomatic resolution between the third and sixth months of antibiotic therapy, as well as an endoscopic and histological improvement. Even in these patients, there was maintenance of periodic acid-Schiff positive macrophages, although in small number and with a more diffuse distribution. Gastrointestinal symptoms predominated in cases of clinical relapse. CONCLUSIONS: The gastrointestinal and general symptoms as well as the arthralgia were the predominant manifestations. The majority of patients showed clinical and endoscopic improvement in response to treatment with trimethoprim-sulfamethoxazole. However, in these cases periodic acid-Schiff positive macrophages can remain for years. Thus, in the absence of clinical deterioration, the presence of these structures is not indicative ofactive disease.
Assuntos
Antibacterianos/uso terapêutico , Combinação Trimetoprima e Sulfametoxazol/uso terapêutico , Doença de Whipple/tratamento farmacológico , Doença de Whipple/patologia , Adulto , Idoso , Biópsia , Feminino , Humanos , Mucosa Intestinal/patologia , Intestino Delgado/patologia , Macrófagos/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
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.