[Autoimmune pancreatitis. An infrequent or underdiagnosed entity? Pathological, clinical and immunological characteristics]. / Pancreatitis autoinmune. 'Una entidad poco frecuente o infradiagnosticada? Características anatomopatológicas, clínicas e inmunológicas.

In the last few years, reports of pancreatic inflammatory diseases caused by immunological mechanisms and with good response to steroid treatment have increased. Although this entity has been known by a number of names, at present the most widely accepted is that of autoimmune chronic pancreatitis (ACP). The present report describes the clinical, immunological, morphological, functional and pathological characteristics of two patients recently studied at our unit and discusses currently used diagnostic tests. The two patients had a complete response to steroid therapy. In our opinion, ACP is probably underdiagnosed in Spain. The availability of morphological, pathological and serological diagnostic tools developed in recent years will help to precisely determine the epidemiology of this process. Thus, quantification of serum levels of anti-carbonic anhydrase II and IgG4 has greatly contributed to the diagnosis of ACP. These tests should be performed in patients with a possible diagnosis of ACP, those suffering from diabetes mellitus type I with impairment of exocrine function, and those with alcoholic pancreatitis and a poor response to alcohol elimination. Once we are able to diagnose and determine the real prevalence of ACP in our setting, the most appropriate therapy and prognosis of this disease can be established.

Pancreatitis autoinmune. ¿Una entidad poco frecuente o infradiagnosticada? Características anatomopatológicas, clínicas e inmunológicas / Autoimmune pancreatitis. An infrequent or underdiagnosed entity? Pathological, clinical and immunological characteristics

En los últimos años se han descrito cada vez con mayor frecuencia enfermedades inflamatorias pancreáticas provocadas por mecanismos inmunológicos, y que responde a un tratamiento esteroideo. Aunque esta entidad ha recibido multitud de denominaciones, la más aceptada actualmente es la de pancreatitis crónica autoinmune. En este trabajo describimos los rasgos clínicos, inmunológicos, morfológicos, funcionales y anatomopatológicos de 2 casos que hemos tenido la oportunidad de estudiar recientemente, a la vez que se comentan las pruebas diagnósticas utilizables actualmente. Estos pacientes tuvieron una respuesta completa al tratamiento esteroideo. Probablemente, la pancreatitis autoinmune es una entidad infradiagnosticada hasta el momento en España. La disponibilidad de herramientas diagnósticas basadas en la imagen, la histología y la serología establecidas en los últimos años servirá para determinar con exactitud la epidemiología de esta enfermedad. En este sentido, la determinación de los valores de anticuerpos anti-AC II y de IgG4 en suero ha contribuido enormemente a la sospecha diagnóstica de esta enfermedad. Estas pruebas deberían solicitarse en los pacientes que tengan un diagnóstico posible de pancreatitis autoinmune, que sufran una diabetes mellitus tipo I con deterioro de la función exocrina y en los pacientes con pancreatitis alcohólica con una mala respuesta a la supresión del alcohol. Una vez consigamos diagnosticar y conocer la verdadera prevalen cia de esta enfermedad en nuestro entorno, queda pendiente establecer el tratamiento más correcto junto con su pronóstico

In the last few years, reports of pancreatic inflammatory diseases caused by immunological mechanisms and with good response to steroid treatment have increased. Although this entity has been known by a number of names, at present the most widely accepted is that of autoimmune chronic pancreatitis (ACP). The present report describes the clinical, immunological, morphological, functional and pathological characteristics of two patients recently studied at our unit and discusses currently used diagnostic tests. The two patients had a complete response to steroid therapy. In our opinion, ACP is probably underdiagnosed in Spain. The availability of morphological, pathological and serological diagnostic tools developed in recent years will help to precisely determine the epidemiology of this process. Thus, quantification of serum levels of anti-carbonic anhydrase II and IgG4 has greatly contributed to the diagnosis of ACP. These tests should be performed in patients with a possible diagnosis of ACP, those suffering from diabetes mellitus type I with impairment of exocrine function, and those with alcoholic pancreatitis and a poor response to alcohol elimination. Once we are able to diagnose and determine the real prevalence of ACP in our setting, the most appropriate therapy and prognosis of this disease can be establishe