RESUMEN
The overall objective of these guidelines is to provide evidence-based recommendations for the diagnosis and management of immunoglobulin G4 (IgG4)-related digestive disease in adults and children. IgG4-related digestive disease can be diagnosed only with a comprehensive work-up that includes histology, organ morphology at imaging, serology, search for other organ involvement, and response to glucocorticoid treatment. Indications for treatment are symptomatic patients with obstructive jaundice, abdominal pain, posterior pancreatic pain, and involvement of extra-pancreatic digestive organs, including IgG4-related cholangitis. Treatment with glucocorticoids should be weight-based and initiated at a dose of 0.6-0.8 mg/kg body weight/day orally (typical starting dose 30-40 mg/day prednisone equivalent) for 1 month to induce remission and then be tapered within two additional months. Response to initial treatment should be assessed at week 2-4 with clinical, biochemical and morphological markers. Maintenance treatment with glucocorticoids should be considered in multi-organ disease or history of relapse. If there is no change in disease activity and burden within 3 months, the diagnosis should be reconsidered. If the disease relapsed during the 3 months of treatment, immunosuppressive drugs should be added.
Asunto(s)
Enfermedades del Sistema Digestivo/tratamiento farmacológico , Enfermedad Relacionada con Inmunoglobulina G4/tratamiento farmacológico , Quimioterapia de Inducción/normas , Quimioterapia de Mantención/normas , Adulto , Peso Corporal , Niño , Enfermedades del Sistema Digestivo/diagnóstico , Enfermedades del Sistema Digestivo/inmunología , Relación Dosis-Respuesta a Droga , Cálculo de Dosificación de Drogas , Europa (Continente) , Medicina Basada en la Evidencia/métodos , Medicina Basada en la Evidencia/normas , Gastroenterología/métodos , Gastroenterología/normas , Glucocorticoides/administración & dosificación , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/inmunología , Inmunosupresores/administración & dosificación , Quimioterapia de Inducción/métodos , Quimioterapia de Mantención/métodos , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
Pancreatic neuroendocrine tumors (PNETs) are very rare, accounting for 1-2% of all pancreatic neoplasms. They are classified into functioning and non-functioning and their behavior varies widely from benign to highly malignant. For their investigation, a variety of anatomical and functional imaging methods are available. Anatomical methods include computed tomography (CT), magnetic resonance imaging, and ultrasonography. Functional methods include scintigraphy and positron emission tomography (PET). A combination of anatomical and morphological methods results in the so-called hybrid imaging, such as PET/CT. We herein discuss the currently available imaging modalities for the investigation of PNETs and, more specifically, their applications in tumor detection and staging as well as in choice of therapy, imaging follow up and prediction of response, with emphasis on the recent developments.