ABSTRACT
Objective To analyze the clinical features of patients with IgG4-associated biliary and pancreatic diseases,and to improve the understanding of these diseases.Methods Nine cases of patients with IgG4-associated pancreatitis and cholangitis were analyzed retrospectively,which included clinical manifestations,serological examination,imaging test,pathology,treatment and prognosis.Results Of the 9 patients,8 were male,1 was female,the average age was 61 years old.Four cases were presented with jaundice,2 cases with jaundice and abdominal pain,2 cases with abdominal pain,and 1 case with diabetes.Serum IgG4 levels were 3.46-21.3 g/L (mean 9.743 g/L) in 6 cases,and which were higher than normal value,3 patients did not undergo blood test of IgG4.Nineteen auto-antibodies (including ANCA) were all negative in 9 cases.CA19-9 was increased in 4 cases.CT manifestations showed 5 cases with low density pancreas mass (2 cases with biliary tract dilatation);1 case had pancreas swelling with dilatation of bile duct,and 2 cases with bile duct dilatation only,and CT findings in 1 case were negative.Four patients underwent EUS-FNA,and EUS features included hypoechoic lesions without peripancreatic lymph nodes.FNA results indicated 2 cases with IgG4 related chronic inflammation,2 cases with chronic inflammation with negative IgG4.Seven cases were confirmed to have IgG4 related pancreatitis,and 2 cases with IgG4 related cholangitis.Six patients received glucocorticoid treatment,and the dose ranged from 8-40 mg;3 patients underwent surgery and the surgical pathology indicated IgG4 positive plasma cell.Follow-up showed the serum IgG4 returned to normal,clinical symptoms improved remarkably,and pancreatic mass decreased.Conclusions IgG4 positive plasma cell infiltration is the main feature of IgG4-associated cholangitis and pancreatitis.The diagnosis should combine image,serology with pathology.Glucocorticoid is an effective treatment.
ABSTRACT
Cualquier enfermedad que lleve a la alteración del flujo biliar o del metabolismo de las sales biliares se traduce en colestasis. Son múltiples las causas que pueden producirla, sea por su localización anatómica intrahepática o extrahepática, agudas o crónicas, con o sin lesión hepatocelular acompañante, o primarias o secundarias, por lo que resultan numerosas las entidades que deben ser consideradas como parte del diagnóstico diferencial de las enfermedades colestásicas y que plantean un gran reto diagnóstico tanto para el clínico, como para el patólogo (1). En el presente estudio se plantea una aproximación diagnóstica basada en patrones histológicos, haciendo énfasis en las enfermedades colestásicas crónicas del adulto, en próximos estudios se tratarán las de la población pediátrica.
Any disease that leads to impaired bile flow or impaired bile salt metabolism results in cholestasis. There are several causes of the disease related to intrahepatic or extrahepatic anatomical locations, to whether the disease is acute or chronic, to whether or not hepatocellular damage occurs, and to whether or not the condition is primary or secondary. The large number of entities that must be considered in the differential diagnosis of cholestatic diseases poses a major diagnostic challenge for both the clinician and the pathologist (1). This article establishes a diagnostic approach based on histologic patterns which emphasizes adult chronic cholestatic diseases. The next article will focus on the pediatric population.