Asunto(s)
Dolor Abdominal , Endometriosis , Humanos , Femenino , Dolor Abdominal/diagnóstico , Dolor Abdominal/etiologíaAsunto(s)
Dolor Abdominal/etiología , Fiebre/etiología , Linfangiectasia Intestinal/complicaciones , Anomalías Linfáticas/complicaciones , Dolor Abdominal/diagnóstico , Adulto , Biopsia , Dieta con Restricción de Grasas , Endoscopía Gastrointestinal , Fiebre/diagnóstico , Humanos , Linfangiectasia Intestinal/diagnóstico , Linfangiectasia Intestinal/dietoterapia , Anomalías Linfáticas/diagnóstico , Anomalías Linfáticas/cirugía , Masculino , Recurrencia , Tomografía Computarizada por Rayos X , Triglicéridos/administración & dosificaciónAsunto(s)
Coristoma/complicaciones , Coristoma/diagnóstico , Intususcepción/etiología , Intususcepción/patología , Enfermedades del Yeyuno/etiología , Enfermedades del Yeyuno/patología , Páncreas , Adulto , Biopsia , Coristoma/patología , Coristoma/cirugía , Enteroscopía de Doble Balón , Histocitoquímica , Humanos , Enfermedades del Yeyuno/cirugía , Masculino , Microscopía , Membrana Mucosa/patología , Radiografía Abdominal , Tomografía Computarizada por Rayos X , Resultado del TratamientoAsunto(s)
Dolor Abdominal/etiología , Enfermedad Celíaca/complicaciones , Diarrea/etiología , Gastritis/complicaciones , Náusea/etiología , Pérdida de Peso , Adulto , Antiulcerosos/uso terapéutico , Biopsia , Enfermedad Celíaca/diagnóstico , Enfermedad Celíaca/terapia , Dieta Sin Gluten , Duodeno/patología , Femenino , Gastritis/diagnóstico , Gastritis/terapia , Humanos , Omeprazol/uso terapéuticoRESUMEN
BACKGROUND & AIMS: Autoimmune enteropathy is a rare cause of intractable diarrhea associated with circulating gut autoantibodies and a predisposition to autoimmunity. It is rarely observed in adults, with only 11 cases reported to date. METHODS: Fifteen adults with autoimmune enteropathy were identified at the Mayo Clinic, Rochester, from May 2001-June 2006. The demographic, clinical, and treatment data were abstracted from their records. RESULTS: The study population was 87% white, 47% female, with median age of 55 years (interquartile range, 42-67 years). All patients had protracted diarrhea, weight loss, and malnutrition. Celiac disease was excluded by lack of response to gluten-free diet or absence of the celiac disease susceptibility HLA genotypes. Fourteen patients were tested for gut epithelial cell antibodies, and 93% were positive for anti-enterocyte and/or anti-goblet cell antibodies. Predisposition to autoimmune diseases was noted in 80%, as indicated by a variety of circulating autoantibodies. Small intestinal histopathologic findings included subtotal villous atrophy and lymphoplasmacytic infiltration in the lamina propria with relatively few surface intraepithelial lymphocytes. T-cell receptor gene rearrangement studies were negative in all cases. Immunosuppressive therapy was required in 93% of cases. Clinical improvement was noted in 60% after 1-8 weeks of steroid therapy. CONCLUSIONS: Autoimmune enteropathy is a heterogeneous disease and should be considered in the differential diagnosis of malabsorption and small bowel villous atrophy. The presence of gut epithelial cell antibodies can help confirm the diagnosis. No single agent is unequivocally effective in inducing remission, and immunosuppressive therapy is required in most cases.
Asunto(s)
Anticuerpos/sangre , Enfermedades Autoinmunes/diagnóstico , Enfermedades Intestinales/diagnóstico , Intestino Delgado/patología , Adulto , Anciano , Atrofia , Enfermedades Autoinmunes/tratamiento farmacológico , Biopsia , Diarrea/etiología , Endoscopía del Sistema Digestivo , Enterocitos/inmunología , Femenino , Estudios de Seguimiento , Células Caliciformes/inmunología , Humanos , Inmunosupresores/uso terapéutico , Enfermedades Intestinales/tratamiento farmacológico , Enfermedades Intestinales/inmunología , Linfocitos/patología , Masculino , Desnutrición/etiología , Persona de Mediana Edad , Estudios Prospectivos , Estudios Retrospectivos , Pérdida de PesoRESUMEN
BACKGROUND: A 72-year-old man was referred for evaluation of dysgeusia, diarrhea and anorexia. 3 months prior he began to experience taste changes, a decline in appetite and 3-7 loose, non-bloody stools per day. Nausea and lower abdominal cramping subsequently developed and he lost 22.68 kg in weight. His past medical history included atrial fibrillation treated with anticoagulation and digoxin. In the past, he had experienced markedly increased levels of triglycerides and was being treated for this condition with a lipid-lowering agent. There was no family history of colorectal neoplasia or IBD. He was a non-smoker and did not drink alcoholic beverages. INVESTIGATIONS: Medical history, physical examination, laboratory evaluation (including 72 h stool collection), upper endoscopy, colonoscopy and histologic analysis of biopsy samples. DIAGNOSIS: Cronkhite-Canada syndrome. MANAGEMENT: Prednisone (40 mg orally once daily, eventually tapered to 10 mg orally once daily), a histamine-2-receptor blocker and oral micronutrient supplementation (iron, vitamins A, E and D and a multivitamin). Removal of all visible polyps from the anal verge to 25 cm endoscopically by snare polypectomy or with hot biopsy forceps, followed by subtotal colectomy with end-to-side ileorectostomy.