ABSTRACT
Introduction: Autoimmune pancreatitis (AIP) is one of the etiologies of chronic pancreatitis, which is classified in two subtypes: type 1 that is part of a fibroinflammatory systemic disease associated with IgG4; and type 2, confined to pancreatic tissue without IgG4 association. Both forms typically present as abdominal pain associated with obstructive jaundice. Radiologically it is characterized by diffuse or focal enlargement of the pancreas, becoming essential to differentiate from pancreatic cancer. Case report: We report the case of a 74 year-old patient who presented obstructive jaundice and abdominal pain, images with diffusely increased pancreatic volume and elevated serum IgG4. She was treated with prednisone with an excellent clinical and laboratory response. Conclusion: AIP is a disease with high clinical suspicion, well-established diagnostic criteria and standardized treatment, showing a high rate of response to treatment of first and second line. AIP pancreatitis diagnosis should be considered facing over patients with acute pancreatitis, chronic pancreatitis or pancreatic cancer.
Introducción: La pancreatitis autoinmune (PAI) es una causa de pancreatitis crónica que se clasifica en dos subtipos: la tipo 1, que es parte de una enfermedad fibroinflamatoria sistémica asociada a IgG4; y la tipo 2, limitada al tejido pancreático y sin asociación a IgG4. Ambas se presentan típicamente como un cuadro de dolor abdominal asociado a ictericia obstructiva. Imagenológicamente se caracteriza por aumento de volumen difuso o focal del páncreas, haciéndose indispensable hacer el diagnóstico diferencial con el cáncer de páncreas. Caso clínico: Presentamos el caso de una paciente de 74 años con ictericia obstructiva y dolor abdominal, imágenes con aumento difuso de volumen pancreático e IgG4 plasmática elevada. Se trata con prednisona con excelente respuesta clínica y de laboratorio. Conclusión: La PAI es un cuadro de alta sospecha clínica, con criterios diagnósticos bien establecidos y tratamiento estandarizado, presentando una alta tasa de respuesta a tratamiento de primera y segunda línea. El diagnóstico de PAI debe ser considerado al enfrentar un paciente con pancreatitis aguda, crónica o cáncer de páncreas.
Subject(s)
Humans , Female , Aged , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , Immunoglobulin G , Pancreatitis/diagnosis , Pancreatitis/immunology , Chronic DiseaseABSTRACT
Objective To analyze the clinical characteristic of Chinese autoimmune pancreatitis (AIP) patients.Methods All clinical data of 81 patients with a diagnosis of AIP in Shanghai Changhai Hospital from February 2005 to May 2012 were analyzed.Results The sex ratio was 7.1∶1 and the mean age was (57± 12) years old in 81 patients with AIP.Obstructive jaundice was the initial symptom in 51.9％ (42/81) patients.In patient receiving CT,focal and diffuse type accounted for 45 and 35 patients.respectively,and pseudocyst was the main manifestation in 1 patient,biliary tract was involved in 59(72.8％ ) patients,dilatation of main pancreatic duct was observed in 5 ( 11.1％ ) patients.In patients receiving PET-CT,diffuse increased Flourine-18 FDG uptake by the pancreas was found in 11 patients,focal increased uptake in 2patients,and significant extra-pancreatic uptake was found in 5 patients.The positive rate of serum IgG4,CA19-9,ss DNA,anti-nuclear antibody and ds-DNA antibody was 94.6％ (53/81),54.4％ (37/68),14.3％ (4/28),10.7％ (3/28),7.1％ (2/28),respectively.The pathological findings of H-E staining and IgG4 immunohistochemical analysis in 20 patients were consistent with lymphoplasmacytic sclerosing pancreatitis.Conclusions Type 1 AIP is the main subtype of AIP in China.Combining clinical symptoms,extra-pancreatic manifestations,imaging or nuclear medicine findings,serology,cytology or histology can effectively increase the correct diagnosis rate of AIP.
ABSTRACT
Lymphoplasmacytic sclerosing pancreatitis (LPSP) is a rare entity that has been described under many different names; LPSP is an autoimmune form of chronic pancreatitis. LPSP may simulate a neoplastic process both clinically and radiologically. We report a case of LPSP with pancreatic adenocarcinoma. A 70-year-old woman was admitted to our hospital for evaluation of pancreatic duct dilatation. The CA 19-9 level was normal and the antinuclear antibody titer was negative. An abdominal CT revealed a low density nodule, 8 mm in size, in the body of the pancreas with parenchymal atrophy and mild dilatation of the main pancreatic duct in the body and tail portion. Endoscopic retrograde cholangiopancreaticography demonstrated a stricture of the main pancreatic duct in the body of the pancreas and mild dilatation of the upstream duct. She underwent subtotal pancreatectomy and splenectomy. The results of the pathologic examination of the resected tissue included pancreatic ductal adenocarcinoma with pancreatic intraepithelial neoplasia in the background of lymphoplasmacytic sclerosing pancreatitis.
Subject(s)
Aged , Female , Humans , Adenocarcinoma , Antibodies, Antinuclear , Atrophy , Constriction, Pathologic , Dilatation , Pancreas , Pancreatectomy , Pancreatic Ducts , Pancreatitis , Pancreatitis, Chronic , SplenectomyABSTRACT
Lymphoplasmacytic sclerosing pancreatitis, also referred to as autoimmune pancreatitis, is a benign disease characterized by irregular narrowing of the pancreatic duct, swelling of the pancreatic parenchyma, lymphoplasmacytic infiltration and fibrosis. A few cases with locally affected lesions show features similar to cancer. Lymphoplasmacytic sclerosing pancreatitis is the most common benign disease in patients undergoing Whipple resection for a presumed pancreatic malignancy. We report a case of lymphoplasmacytic sclerosing pancreatitis diagnosed after surgery in a patient presenting with obstructive jaundice, with a review of the literature.