ABSTRACT
La pancreatitis autoinmune es una enfermedad recientementecaracterizada y que en la actualidad constituye un reto diagnósticoespecialmente su diferenciación con el cáncer de páncreas. Suevolución a largo plazo es poco conocida, presentándose un casoestudiado a lo largo de 14 años y mostrando su evolución clínica,bioquímica y morfológica.Paciente mujer de 54 años que debuta con un cuadro de ictericiaobstructiva y molestias abdominales inespecíficas y constataciónen la TAC de un aumento de la cabeza del páncreas, todoello sugestivo de neoplasia de páncreas. Fue intervenida evidenciándoseun aumento difuso de todo el páncreas descartándosemalignidad intraoperatoriamente, realizando únicamente colecistectomíay coledocoduodenostomía, quedando diagnosticada entoncescomo pancreatitis crónica. Durante los años posterioresfueron apareciendo diferentes procesos autoinmunes como asma,sialoadenitis y colangitis esclerosante secundaria, así como episodiosrecurrentes de ictericia e insuficiencia pancreática endocrinay exocrina. La aparición de estas complicaciones y la detección deniveles séricos elevados de IgG4 y de anticuerpos antianhidrasacarbónica II condujo a la reevaluación de la histología inicial concluyendofinalmente con el diagnóstico de pancreatitis autoinmuneal evidenciarse una infiltración linfocitaria y plasmacitariaIgG4+, así como fibrosis y flebitis obliterativa. En los últimos añosse ha añadido a las anteriores complicaciones una fibrosis retroperitonealcon hipertensión portal, varices esofágicas y esplenomegalia
Autoimmune pancreatitis is a recently characterized diseasethat still constitutes a diagnostic challenge, especially regarding differentialdiagnosis from neoplasia. Long-term outcome is poorlyknown. We herein report a case of a patient with autoimmunepancreatitis and 14 years of follow-up, and show its clinical, biochemical,and morphological characteristics.A 54-year-old female presented with obstructive jaundice andabdominal tenderness, as well as a mass at the pancreatic head ona CT scan, suggestive of pancreatic neoplasia. Surgery showed anincrease of the whole pancreas, malignancy was intraoperativelyruled out, and a cholecystectomy and choledochoduodenostomywere carried out. The diagnosis was chronic pancreatitis. Over thefollowing years different autoimmune complications developed, includingasthma, salivary gland swelling, and sclerosing cholangitis,as well as recurrent episodes of jaundice, and exocrine and endocrinepancreatic failure. The development of these complicationscombined with the demonstration of high serum levels ofIgG4 and carbonic anhydrase II led to a re-evaluation of the initialhistology of the pancreas, leading to a final diagnosis of autoimmunepancreatitis: IgG4+ lymphoplasmacytic infiltrates, fibrosis,and obliterative phlebitis. New complications developed during thelast few years: retroperitoneal fibrosis with portal hypertension,esophageal varices, and splenomegaly
Subject(s)
Autoimmune Diseases/complications , Granuloma, Plasma Cell/complications , Hypertension, Portal/complications , Retroperitoneal Fibrosis/complications , Autoimmune Diseases/diagnosis , Autoimmune Diseases , Autoimmune Diseases/surgery , Splenomegaly/etiology , Time Factors , Tomography, X-Ray Computed , Cholangiography , Cholangitis, Sclerosing/etiology , Cholecystectomy , Chronic Disease , Diagnosis, Differential , Esophageal and Gastric Varices/etiology , Follow-Up StudiesABSTRACT
Autoimmune pancreatitis is a recently characterized disease that still constitutes a diagnostic challenge, especially regarding differential diagnosis from neoplasia. Long-term outcome is poorly known. We herein report a case of a patient with autoimmune pancreatitis and 14 years of follow-up, and show its clinical, biochemical, and morphological characteristics. A 54-year-old female presented with obstructive jaundice and abdominal tenderness, as well as a mass at the pancreatic head on a CT scan, suggestive of pancreatic neoplasia. Surgery showed an increase of the whole pancreas, malignancy was intraoperatively ruled out, and a cholecystectomy and choledochoduodenostomy were carried out. The diagnosis was chronic pancreatitis. Over the following years different autoimmune complications developed, including asthma, salivary gland swelling, and sclerosing cholangitis, as well as recurrent episodes of jaundice, and exocrine and endocrine pancreatic failure. The development of these complications combined with the demonstration of high serum levels of IgG4 and carbonic anhydrase II led to a re-evaluation of the initial histology of the pancreas, leading to a final diagnosis of autoimmune pancreatitis: IgG4+ lymphoplasmacytic infiltrates, fibrosis, and obliterative phlebitis. New complications developed during the last few years: retroperitoneal fibrosis with portal hypertension, esophageal varices, and splenomegaly.
