A case of autoimmune pancreatitis complicated with immune thrombocytopenia]

A 80-year old man was referred to our hospital because of an elevation of serum amylase level. Diffuse enlargement of the pancreas was detected by abdominal computed tomography, and also diffuse narrowing of the main pancreatic duct was revealed using endoscopic retrograde cholangiopancreatography. The serum level of IgG was elevated to 3450mg/dl. Besides, on the 10th hospital day, petechia developed and the platelet level decreased to 1.5 x 10(4)/microl. The platelet-associated IgG, antiplatelet antibody and antinuclear antibody in serum were positive. The levels of serum complements were low. From all these findings the patient was diagnosed as autoimmune pancreatitis complicated with immune thrombocytopenia. The treatment with prednisolone was started, which was effective on each disease. The medication was suspended a year ago, and so far there is no data suggesting the recurrence of autoimmune pancreatitis or immune thrombocytopenia.     

自身免疫性胰腺炎1例报道

1临床资料患者,男,59岁。因上腹胀痛17个月,加重4 d于2010年8月14日入院。患者缘于17个月前无明显诱因出现上腹持续性胀痛,向背部放散,伴乏力,皮肤瘙痒,随就诊于长春市208医院,化验肝功能,转氨酶及胆红素轻度升高,疑似肝炎给予保肝降酶治疗,肝功能改善     

Involvement of the biliary system in autoimmune pancreatitis: a follow-up study.

BACKGROUND & AIMS: The aim of this study was to define the bile duct changes associated with autoimmune pancreatitis. METHODS: Eight patients with autoimmune pancreatitis were followed for a mean of 4 years. The clinical features of these patients, including extrapancreatic bile duct changes, were examined by using biochemical parameters and several imaging modalities. Pathologic features of the pancreas and liver were examined by using the biopsy specimens of 7 patients. RESULTS: Diffuse or focal narrowing of the main pancreatic duct was observed in all patients. Histologic examination of the pancreas showed lymphoplasmacyte infiltration with severe fibrosis and acinar cell depletion. In 6 patients extrapancreatic bile duct changes such as stricture of the bile duct at hilus or intrahepatic area were observed. In 2 patients abnormalities in the bile duct and pancreas were detected simultaneously at diagnosis, and changes in the bile duct were observed later in 4 patients. Lymphoplasmacyte infiltration and fibrosis were observed in the portal area of all 7 liver biopsy samples. Five of the patients with bile duct changes received steroid therapy, and the pathological changes improved. CONCLUSIONS: Extrapancreatic bile duct changes are frequently associated with autoimmune pancreatitis. Similar pathogenic mechanism might produce the biliary tract and pancreatic abnormalities in autoimmune pancreatitis resulting in a similar histopathology in the liver and pancreas and response to steroid therapy.     

Treating patients with autoimmune pancreatitis: results from a long-term follow-up study.

BACKGROUND: Steroid therapy is currently common treatment for autoimmune pancreatitis (AIP); however, indications of steroid therapy have yet to be established, and the clinical course after steroid therapy is unknown. METHODS: A total of 23 patients with AIP were subdivided into 4 groups according to the initial treatments undertaken. They were treated with pancreatoduodenectomy on suspicion of pancreatic tumor in 6 patients, choledochoduodenostomy with pancreatic biopsy in 4 patients, supportive therapy in 3 patients, and steroid therapy in 10 patients. Clinical course of AIP in each group was examined. RESULTS: Prognosis of the AIP patients is almost good except for the 2 patients who progressed to pancreatic insufficiency after resection. Two patients without jaundice improved spontaneously. Steroid therapy was effective in all patients treated, but pancreatic atrophy developed in 5 of these patients. Steroid therapy improved insulin secretion and glycemic control in 4 of 7 diabetes mellitus (DM) patients. CONCLUSION: To avoid futile surgery, in relatively elderly male patients with obstructive jaundice suggestive of pancreatic carcinoma, preoperative clinical suspicion of AIP is mandatory. Indications of steroid therapy for AIP are thought to be obstructive jaundice due to stenosis of the bile duct, other associated systemic autoimmune, and DM coincidental with AIP.     

自身免疫性胰腺炎1例报告

<正>自身免疫性胰腺炎(AIP)是与自身免疫损伤相关的慢性胰腺炎症,随着研究的深入,其作为一个全新的临床疾病独立分型受到越来越多的关注,因其免疫球蛋白(Ig)G4水平显著升高,又称为IgG4相关性胰腺炎[1]。目前关于AIP确切的发病机制尚不清楚,有研究报道辅助性T细胞(helper T cell,Th)1、Th2、抗原递呈细胞(APC)、调节性T细胞(Treg)以及多种细胞     

A case report of autoimmune pancreatitis with Mikulicz' s disease and diabetes mellitus]

A 56-year-old man with bilateral swelling of lacrimal glands was admitted to our hospital. He was diagnosed as autoimmune pancreatitis with Mikulicz' s disease presenting the swelling of lacrimal glands, submandibular glands and the pancreas head and tail. Treatment with systemic prednisolone resulted in improvement of the swelling of these glands and pancreas. On the immunohistochemical examination, infiltration of CD4- and CD8-positive T lymphocytes was detected in the lacrimal gland, the submandibular gland, the gall bladder and the pancreas. Infiltration of IgG4-positive plasma cells was detected in the submandibular gland, the gall bladder and the pancreas. These results may suggest the presence of common etiology between autoimmune pancreatitis and Mikulicz' s disease.     

A case of autoimmune pancreatitis with multifocal mass lesions

We present a case of a 73-year-old man with multifocal autoimmune pancreatitis (AIP) in the pancreatic head and tail, and who had undergone sigmoidectomy and rectectomy 28 months before presenting to our department. Upon presentation, his serum IgG4 level was elevated at 267mg/dl, but tumor marker levels were within normal ranges. CT and MRI showed two localized pancreatic masses with delayed enhancement, but endoscopic retrograde pancreatography revealed neither stenosis nor dilatation of the main pancreatic duct. FDG-PET examination showed intense uptake in regions concordant with both tumors. The possibility of atypical AIP was a concern, but malignant tumor could not be clinically or radiologically excluded. Endoscopic ultrasonographic fine-needle aspiration biopsy was performed, but no malignant cells were detected. The patient underwent subsequent distal pancreatectomy. Histological evaluation of the tumors showed the presence of many IgG4-positive plasma cells without any evidence of malignancy.     

A case of pancreatic carcinoma with suspected autoimmune pancreatitis

We present a case of pancreatic carcinoma with strongly suspected coexisting autoimmune pancreatitis (AIP). The patient presented with a chief complaint of icterus and weight loss, and was referred to our institution after a pancreatic lesion was found. Blood test showed elevation of serum bilirubin, hepato-biliary enzyme, glucose and tumor markers, and also high levels of serum IgG4 (344 mg/dl, normal 4.8–105 mg/dl) and anti-DNA antibody (14 IU/ml, normal <6.0 IU/ml). Ultrasonography demonstrated an enlarged pancreas with smooth borders and low internal echo density. Enhanced computed tomography (CT) showed a sausage-shaped pancreas without definitive metastasis to the surrounding lymph nodes and liver. Imaging of the pancreatic duct, including endoscopic retrograde pancreatography (ERP) and magnetic resonance cholangiopancreatography (MRCP), showed stenosis of the main pancreatic duct at the pancreatic head as well as a long segment of narrowing at the body and no dilatation at the tail. Tissues from these stenotic sites and open biopsy from pancreatic body showed infiltrating adenocarcinoma and dense fibrosis. To date, only a small number of reports have described pancreatic carcinoma accompanied with AIP. It is important to confirm diagnosis with histology in cases of suspicious autoimmune pancreatitis, even when the clinical images are compatible with AIP.     

A case of autoimmune pancreatitis associated with ulcerative colitis

A 31-year-old man was admitted to our hospital because of upper abdominal pain. He had been diagnosed with ulcerative colitis (UC) at age 28, but the disease has been in remission since then. On admission, he had slight fever, abdominal pain, and bloody stools six times a day, and the serum levels of pancreatic enzyme and IgG4 were elevated. Diffuse enlargement of the pancreas was detected by abdominal computed tomography; furthermore, narrowing of the main pancreatic duct was revealed using endoscopic retrograde cholangiopancreatography. Based on these findings, he was given a diagnosis of autoimmune pancreatitis (AIP) associated with UC. Both diseases improved without using steroids. After discharge, he has not had any recurrence of AIP or UC despite not being on steroid treatment, although the serum IgG4 level has shown a slight tendency to elevate.     

The long-term follow-up results after steroid treatment in patients with autoimmune chronic pancreatitis]

BACKGROUND/AIMS: Autoimmune chronic pancreatitis (AIP) is a clinically attractive entity because of its dramatic response to steroid therapy. But the long-term results after steroid therapy have not been reported yet in Korea. The purpose of this study was to assess the long-term results and prognosis after steroid therapy in patients with AIP. METHODS: We retrospectively analyzed the clinical, radiologic, and laboratory features and evaluated clinical outcomes in 19 patients with AIP who have been treated with oral corticosteroid. All patients were initially treated with prednisolone (30-40 mg/d) for 1 or 2 months. After the confirmation of clinical improvement in radiologic imaging and laboratory findings, the daily dose of prednisolone was then gradually tapered by 5-10 mg per month to the maintenance dose (2.5-7.5 mg/d). RESULTS: All the patients showed normalization or marked improvement in symptoms, laboratory and imaging findings after steroid therapy. There were 4 cases (21%) of recurrence during the mean follow-up period of 27 months. All the patients with recurrence responded to oral steroid again. Among the 10 patients with diabetes mellitus, seven patients were able to stop or reduce the medication for diabetes after completion of steroid therapy. The biliary stents were additionally inserted in 10 patients who showed distal common bile duct stricture and obstructive jaundice. The accompanying autoimmune diseases were also improved with oral corticosteroid. CONCLUSIONS: Steroid therapy is very effective for AIP and is also effective in the cases of recurrence. A definitive protocol of steroid therapy for AIP should be established in the future.