Portal Hypertension, Systemic Lymphadenopathy And Splenomegaly Associated With Autoimmune Pancreatitis: A Case Report With Literature Review

1.Background and ObjectiveAutoimmune pancreatitis is a rare form of chronic pancreatitis, first described in 1961 as'primary inflammatory sclerosis of the pancreas' by Sarles H et al. It was also called lymphoplasmacytic sclerosing pancreatitis, chronic sclerosing pancreatitis and nonalcoholic duct destructive chronic pancreatitis before. An elevated serum IgG4 level is a characteristic finding in it. It has been reported that this disease is associated with many other disease, such as retroperitoneal fibrosis, sclerosing cholangitis and sialoadenitis. We herein report an interesting case of a 45-year-old male presented with haematemesis, melena and fever, accompanied by hepatosplenomegaly, systemic lymphadenopathy, as well as the performance of diffuse swelling of the pancreas, portal hypertension and multiple enlargement of the retroperitoneal lymph nodes in the abdominal CT scan. It was first considered the lymphoma, but autoimmune pancreatitis to be excluded. The patient did not have a history of viral hepatitis or cirrhosis. Laboratory testing revealed an elevated IgG(3000.0mg/dl). Due to uncontrolled active upper gastrointestinal bleeding, he underwent operation. We found splenomegaly, with a plump pancreas and the integration of the peripheral lymph nodes, so a splenectomy was carried out, with biopsy of the pancreatic tail and parts of the lymph nodes. All the resected tissues were infiltrated by a large number of IgG4-positive plasma cells. Therefore, this patient was diagnosed as having autoimmune pancreatitis associated with portal hypertension, systemic lymphadenopathy and splenomegaly. The patient received no other treatment after splenectomy. In the course of 6 months of follow-up, the patient was free from any symptoms, besides, the serum IgG also decrased to a normal level, the enhanced computed tomography scan showed a normal image of the pancreas, these all suggested good outcome. We speculate splenectomy may be a new method to treat AIR2. Material and MethodA case of AIP with portal hypertension, systemic lymphadenopathy and splenomegaly in Dec 2009 which was from the first affiliated hospital of Zhejiang University, were reported and analyzed. Simultaneously, we searched and reviewed the literatures related with AIP in the PubMed database of the National Library of Medicine online, Wanfang database system, VIP information database.3. Results and ConclusionsThe onset age, clinical manifestation, laboratory examination, image studies and pathological examination of our case showed the typical morphologic features of AIP. After 15 months follow-up, there was no discomfort with the patient. The serum detection of IgG, IgM, IgA, C3, C4 were all normal, while the examination of autoantibodies was almost the same as before. The quantitative analysis of the urine about the light chainλandκalso revealed a normal level. Besides, superficial lymph nodes ultrasonography revealed that lymphadenopathy disappeared only except the left axillary lymph nodes (with the biggest measured at 1.6*0.9cm). The enhanced computed tomography scan showed a normal image of the pancreas, with no performance of gastric varices or lymphadenopathy in the retroperitoneal.