Autoimmune pancreatitis associated with a large pancreatic pseudocyst

INTRODUCTIONAutoimmune pancreatitis (AIP) is a benign disease that responds well to steroid treatment. Characteristics in-clude radiological evidence of an irregular narrowing of the pancreatic main duct and a diffuse enlargement of the pancreas, together…     

Isolated intrapancreatic Ig G4-related sclerosing cholangitis

Immunoglobulin G4-related sclerosing cholangitis(Ig G4-SC) is frequently associated with type 1 autoimmune pancreatitis(AIP). Association with AIP can be utilized in the diagnosis of Ig G4-SC. However, some cases of Ig G4-SC are isolated from AIP, which complicates the diagnosis. Most of the reported cases of isolated Ig G4-SC displayed hilar biliary strictures, whereas isolated Ig G4-SC with intrapancreatic biliary stricture is very rare. Recently, we have encountered 5 isolated intrapancreatic Ig G4-SC cases that were not associated with AIP, three of which were pathologically investigated after surgical operation. They all were males with a mean age of 74.2 years. The pancreas was not enlarged in any of these cases. No irregular narrowing of the main pancreatic duct was found. Bile duct wall thickening in lesions without luminal stenosis was detected by abdominal computed tomography in all five cases, by endoscopic ultrasonography in two out of four cases and by intraductal ultrasonography in all three cases. In three cases, serum Ig G4 levels were within the normal limits. The mean serum Ig G4 level measured before surgery was 202.1 mg/d L(4 cases). Isolated intrapancreatic Ig G4-SC is difficult to diagnose, especially if the Ig G4 level remains normal. Thus, this type of Ig G4-SC should be suspected in addition to cholangiocarcinoma and pancreatic cancer if stenosis of intrapancreatic bile duct is present.     

Biliopancreatic fistula with portal vein thrombosis caused by a pancreatic pseudocyst

We encountered a very rare case of biliopancreatic fistula with portal vein thrombosis caused by pancreatic pseudocyst. A 57-year-old man was referred to our hospital because of abdominal pain, obstructive jaundice, and portal vein thrombosis due to acute pancreatitis. Computed tomography showed a 7-cm-diameter pseudocyst around the superior mesenteric vein extending towards the pancreatic head, dilatation of the intrahepatic bile duct, and portal vein thrombosis. Endoscopic retrograde pancreatography revealed a main pancreatic duct with a pseudocyst communicating with the common bile duct. After pancreatic sphincterotomy, a 7-F tube stent was endoscopically placed into the pseudocyst. However, a 6-F nasobiliary tube could not be inserted into the bile duct because the fistula had a tight stenosis. Subsequently, the patient’s abdominal pain improved, the pancreatic cyst disappeared, and the serum amylase level normalized. Two months after the endoscopic retrograde cholangiopancreatography, percutaneous transhepatic biliary drainage was required because the patient’s jaundice became aggravated. Two weeks after the choledochojejunostomy, the patient left the hospital in good condition. A follow-up computed tomography showed cavernous transformation of the portal vein and no pancreatic pseudocyst. The patient remains asymptomatic for 2 years and 7 months after surgery. Biliary drainage may be necessary for biliopancreatic fistula with obstructive jaundice in addition to pancreatic cyst drainage. Biliopancreatic fistula can be treated by endoscopic procedure in some cases; however, surgical treatment should be required in cases that are impossible to insert a biliary stent because of hard stricture.     

Synchronous cancer of the biliary tract and pancreas associated with anomalous arrangement of the pancreaticobiliary ductal system.

A 58-year-old man on abdominal ultrasonography and CT had an irregularly elevated lesion at the neck of the gallbladder and a cyst of approximately 6.5 cm in diameter at the pancreatic tail. Percutaneous transhepatic cholangiography revealed a 2-cm shadow defect at the neck of the gallbladder and an irregular, translucent 30 x 12 mm lesion in the intrapancreatic bile duct. Total pancreatectomy and extended cholecystectomy with regional lymph node dissection was performed. An anomalous arrangement of the pancreaticobiliary ductal system (AAPBD) was demonstrated by postoperative contrast radiography of resected specimen. The lesions of the gallbladder and common bile duct were papillary adenocarcinoma. In addition, papillary adenocarcinoma was limited almost entirely to the mucosal layer of the main pancreatic duct and its branches, from the junction of the common bile duct and pancreatic duct to the pancreatic tail. The three tumors were not continuous. The cyst at the pancreatic tail was a pseudocyst. This case represents synchronous cancer of the gallbladder, common bile duct, and pancreas associated with AAPBD.     

A case of autoimmune pancreatitis with metachronous appearance of idiopathic thrombocytopenic purpura

Autoimmune pancreatitis (AIP) is often associated with systemic disorders, but a case accompanied with idiopathic thrombocytopenic purpura (ITP) is very rare. A 67-year-old man was referred to our institution with complaints of abdominal pain and jaundice. Multiple images showed diffuse enlargement of the pancreas, narrowing of the main pancreatic duct, stenosis of the lower common bile duct and thickness of the anterior wall of the abdominal aorta. Serum levels of IgG and IgG₄ were elevated and a diagnosis of AIP was made based on the Japanese criteria. The pancreatic lesion and serum markers responded well to steroid therapy. A year after withdrawal of steroids, the peripheral blood platelet levels gradually decreased to 5.8 × 10⁴/μl. With elevated serum PAIgG levels and exclusion of other causes, we diagnosed ITP. Immediately after restarting steroid therapy, the platelet level in the peripheral blood recovered.     

Inflammatory pseudotumors of the pancreas and liver with infiltration of IgG4-positive plasma cells

Recently, it has been reported that autoimmune pancreatitis (AIP) can be complicated with various extrapancreatic lesions. Here, we report a very rare case of pancreatic and hepatic inflammatory pseudotumor (IPT) with the infiltration of IgG4-positive plasmacytes. The patient showed pancreatic and hepatic masses with elevated levels of serum IgG4. Endoscopic retrograde cholangiopancreatography revealed narrowing of the intrapancreatic bile duct. Fluorine-18fluorodeoxyglucose positron emission tomography suggested pancreatic cancer with hepatic metastasis. Histopathologic findings showed fibrosis and infiltration of IgG4-positive plasmacytes, suggesting IPT. The present case suggests a possible common mechanism in the development of AIP and IPT of the liver.     

Clinical difficulties in the differentiation of autoimmune pancreatitis and pancreatic carcinoma

OBJECTIVES: Autoimmune pancreatitis (AIP) is a newly described entity with characteristic clinical, histologic, and morphologic changes, in which autoimmune mechanisms are involved in pathogenesis. However, difficulties can arise in the differentiation of AIP from pancreatic carcinoma. The aims of this study were to clarify clinical and radiologic features of AIP to elucidate areas of diagnostic confusion. METHODS: Clinical, serologic, and radiologic findings were evaluated in 17 patients with AIP. RESULTS: All 17 patients were initially suspected to have pancreatic carcinoma. The patients were predominantly elderly men who frequently presented with jaundice but without features of acute pancreatitis. Elevation of serum gamma-globulin and IgG along with the presence of autoantibodies were usually evident, whereas serum tumor markers were elevated in 54% of cases. Stenosis of the bile duct was detected in 94% of cases. Diffuse enlargement of the pancreas and irregular narrowing of the main pancreatic duct were radiologically characteristic; however, segmental swelling and narrowing were detected in seven and two patients, respectively. In segmental cases, neither atrophy of the distal pancreas nor marked upstream dilation of the distal main pancreatic duct was observed.Angiographic abnormalities occurred in 54% of cases. Serologic and radiologic abnormalities showed considerable improvement with steroid therapy. CONCLUSIONS: Frequent stenosis of the bile duct, elevation of serum tumor markers, segmental pancreatic enlargement or narrowing of the main pancreatic duct, and angiographic abnormalities can cause confusion in the differential diagnosis of AIP and pancreatic carcinoma.     

Perforation of a pancreatic pseudocyst induced by abdominal sonography

A fifty-year-old man complained of abdominal pain, nausea, loss of appetite, postprandial vomiting and loss of weight. Abdominal sonography revealed a chronic calcifying pancreatitis with a tubular stenosis of the common bile duct, dilatation of the pancreatic duct and multiple pancreatic duct stones. Distal of the pylorus there was an intramural pseudocyst, which had led to the obstruction of the duodenal lumen. Shortly after the ultrasound examination abdominal pain increased. In addition, an elevation of serum lipase levels was noted, but reclined rapidly the next day and was normalized a few days later. The patient was well the next morning, the symptoms of gastric retention disappeared and he had a good appetite. A CT of the abdomen two days later showed the chronic pancreatitis but could not confirm the pseudocyst and a subsequent repeat sonography revealed only a remnant of the pseudocyst. In conclusion it can be assumed that the intramural pseudocyst ruptured when pressure was applied with the ultrasound transducer to displace interfering colonic gas. Although it cannot be advised as a treatment measure for a pseudocyst, regression of a pseudocyst due to emptying into the gastrointestinal tract has to be expected.     

MRCP and MRI findings in 9 patients with autoimmune pancreatitis

AIM: To evaluate magnetic resonance cholangiopancre-atography (MRCP) findings in conjunction with magnetic resonance (MR) images in autoimmune pancreatitis (AIP) patients. METHODS: Nine patients with AIP underwent MRI, MRCP, endoscopic retrograde cholangiopancreatography (ERCP), computed tomography, and ultrasonography. The MRCP and MR images taken before and after steroid therapy were reviewed and compared with other imaging modalities. The MRCP findings of the AIP cases were compared to those of 10 cases with carcinoma of the head of the pancreas. RESULTS: On MRCP, the narrowed portion of the main pancreatic duct noted on ERCP was not visualized, while the non-involved segments of the main pancreatic duct were visualized. The degree of upstream dilatation of the proximal main pancreatic duct was milder than that seen in cases of pancreatic carcinoma. Stenosis or obstruction of the lower bile duct was detected in 8 patients. MR images showed enlargement of the pancreas with decreased signal intensity on T1-weighted MR images, increased signal intensity on T2-weighted MR images, and, in 3 patients, a hypointense capsule-like rim. After steroid therapy, the previously not visualized portion of the main pancreatic duct was seen, along with improvement of the bile duct stenosis. Pancreatic enlargement decreased, and the abnormal signal intensity on both T1- and T2-weighted MR images became isointense. CONCLUSION: MRCP cannot differentiate irregular narrowing of the main pancreatic duct seen with AIP from stenosis of the main pancreatic duct seen with pancreatic carcinoma. However, MRCP findings in conjunction with MR imaging of pancreatic enlargement that shows abnormal signal intensity on T1- and T2-weighted MR images are useful in supporting a diagnosis of AIP.     

自身免疫性胰腺炎误诊误治17例临床分析

目的 总结自身免疫性胰腺炎(AIP)误诊误治的原因.方法 回顾性分析2005年5月至2010年7月间我院收治的17例有误诊误治经历的AIP患者的临床资料.结果 AIP的主要临床症状包括腹部轻度疼痛13例、进行性黄疸12例、发热6例、体重减轻9例.15例伴随一种或一种以上胰腺外脏器的自身免疫性疾病,包括过敏性鼻炎、颌下淋巴结肿大、颌下腺肿大、过敏性哮喘、类风湿性关节炎、干燥综合征、糖尿病、原发性硬化性胆管炎和自身免疫性肝炎.实验室检查方面,血清球蛋白增高11例、IgG增高14例、γ球蛋白增高13例、抗核抗体阳性13例、抗胰岛素IgG抗体阳性2例.影像学检查方面,胰腺弥漫性肿大15例,伴主胰管弥漫性狭窄或节段性狭窄、胰腺段胆总管狭窄、近端胆管扩张、胆囊增大;胰头、钩突局灶性肿大2例.17例中有13例误诊为胰腺癌,其中4例行胰十二指肠切除术,7例行胆肠吻合术,2例被认为肿瘤晚期失去治疗机会;4例被误诊为普通慢性胰腺炎.确诊AIP后,17例患者均接受糖皮质激素治疗,效果显著.随访6～45个月(平均15个月),4例复发,再次子糖皮质激素治疗效果好.随访期间未发现胰腺癌.结论 AIP 临床特点与胰腺癌相似,加之我国现阶段对AIP认识不足、重视不够,是导致误诊和误治的主要原因.

Abstract：

Objective To summarize the reasons of mis-diagnosis and mis-treatment of autoimmune pancreatitis (AIP). Methods Clinical data of 17 patients with AIP,who were admitted to the hospital from May 2005 to July 2010 and experienced mis-diagnosis and mis-treatment, were retrospectively analyzed. Results The main clinical manifestations included epigastric pain (13 cases),progressive obstructive jaundice (12 cases), fever (6 cases) and weight loss (9 cases). Fifteen patients had extrapancreatic organ involvemnet, including allergic rhinitis, swelling of lymphoglandulae submaxillares, swelling of submaxillary gland, allergic asthma, rheumatoid arthritis, Sjogren syndrome, diabetes mellitus, primary sclerosing cholangitis and autoimmune hepatitis. Of these 17 cases, 11 cases presented with high serum globulin, 14 cases with high serum IgG, 13 cases with high serum γ-globulin, 13 cases with positive anti-nuclear antibody and 2 cases with positive anti-insulin IgG antibody. The abdominal imaging demonstrated that 15 patients had diffuse enlargement of the pancreas with diffuse or segmental narrowing of main pancreatic duct, narrowing of the intrapancreatic common bile duct, dilation of the proximal biliary duct and gallbladder enlargement. Focal enlargement of the pancreas was found in 2 cases. Thirteen cases were misdiagnosed as pancreatic carcinoma. Among them, 4 cases underwent pancreaticoduodenectomy and 7 cases underwent choledochojejunostomy. Two cases were misdiagnosed as end stage of cancer that lost therapeutic chance. Another 4 cases were misdiagnosed as chronic pancreatitis. Steroid therapy was administered in all patients with satisfactory response. All patients were followed-up for 15 months (ranged from 6 months to 45 months), and recurrence was found in 4 cases. Satisfactory response was found in patients treated with steroid for the second time. No pancreatic cancer was found in these patients in the follow up period. Conclusion The main causes of mis-diagnosis and mis-treatment of AIP may be contributed by difficulty in differentiating AIP from pancreatic carcinoma based on clinical manifestations and inadequate knowledge of AIP as well as insufficient attention to AIP in China.     

Autoimmune pancreatitis complicated by an infected pseudocyst

A 53-year-old man was admitted due to upper abdominal pain. He had been diagnosed with autoimmune pancreatitis (AIP) 4 years previously and had been taking steroids for maintenance therapy since that time. Abdominal computed tomography revealed multiple pseudocysts in the head of the pancreas and a dilated pancreatic duct with mottled calcifications around the pseudocyst. Despite the continuation of steroid therapy for 4 months, the size of the pseudocyst increased further and diffuse calcifications developed throughout the pancreas. He was readmitted due to severe abdominal pain and a high fever, and endoscopic drainage of the pancreatic pseudocyst was performed. Two months after the internal drainage, the pseudocyst disappeared and his symptoms subsided. There are few reports concerning AIP complicated by the formation of pseudocysts. We report this case to emphasize that AIP can be complicated by the development of pseudocysts with progression to chronic pancreatitis, accompanied by multiple calcifications.     

A case of pancreatic serous cystadenoma with obstructive jaundice

A 62‐year‐old man with precordial pain and fever consulted a local practitioner. Blood tests revealed jaundice. Acute cholecystitis was diagnosed on ultrasonographic examination, and percutaneous transluminal gall‐bladder drainage was performed. The patient was referred to the Department of Surgery for operation. Imaging studies performed via a drain disclosed compression and stenosis of the lower portion of the common bile duct. A computed tomographic scan showed a multilocular nodule‐like low‐density area measuring 2.0 × 2.0 cm in diameter at the head of the pancreas. Endoscopic retrograde cholangiopancreatography disclosed compression and stenosis of the main pancreatic duct at the head of the pancreas. Angiographic examination revealed encasement of the intrapancreatic branch of the posterior pancreatic arcade, located in the same area as the compression stenosis of the bile duct. The results of imaging studies suggested cancer of the head of the pancreas, and a pancreatoduodenectomy was performed. The resected specimen included a mass measuring 3.5 × 2.7 × 1.7 cm, which was located at the head of the pancreas. On examination of a cut section, the mass was found to consist of small multilocular cysts. The cysts invaded the intrapancreatic bile duct. The histopathological diagnosis was serous cystadenoma.     

Endoscopic approach for diagnosing autoimmune pancreatitis

It is of utmost importance to differentiate autoimmune pancreatitis (AIP) from pancreatic cancer (PC). Segmental AIP cases are sometimes difficult to differentiate from PC. On endoscopic retrograde cholangiopancreatography, long or skipped irregular narrowing of the main pancreatic duct (MPD), less upstream dilatation of the distal MPD, side branches derived from the narrowed portion of the MPD, absence of obstruction of the MPD, and stenosis of the intrahepatic bile duct suggest AIP rather than PC. Abundant infiltration of IgG4-positive plasma cells is frequently and rather specifically detected in the major duodenal papilla of AIP patients. IgG4-immunostaining of biopsy specimens obtained from the major duodenal papilla is useful for supporting a diagnosis of AIP with pancreatic head involvement. On endoscopic ultrasonography (EUS), hyperechoic spots in the hypoechoic mass and the duct-penetrating sign suggest AIP rather than PC. EUS and intraductal ultrasonography sometimes show wall thickening of the common bile duct even in the segment in which abnormalities are not clearly observed with cholangiography in AIP patients. EUS-guided fine needle aspiration, especially EUS-guided Tru-Cut biopsy, is useful to diagnose AIP, as well as to exclude PC.     

Ultrasonographic evaluation in patients with autoimmune-related pancreatitis

Background The concept of autoimmune-related pancreatitis (AIP) has recently been described. It is important to exclude pancreaticobiliary malignancy in patients with AIP who develop distal bile duct strictures. The aim of this study was to evaluate distal common bile duct strictures in AIP patients by endoscopic ultrasonography (EUS), intraductal ultrasonography (IDUS), and contrast-enhanced EUS (ceEUS).Methods Five patients with AIP, encountered from January 2000 through December 2001, underwent EUS, IDUS, and ceEUS, using Levovist as a contrast medium. EUS and ceEUS were used to follow changes in distal bile duct strictures in three of these five patients following a trial of steroid therapy.Results Of the five patients, four had cholestatic biochemical profiles, three were positive for autoantibodies, and three had elevated serum immunoglobulin G (IgG) or IgG4. A diffusely enlarged pancreas, narrowing of the main pancreatic duct (MPD), and strictured common bile duct in the pancreatic head were features common to all patients. Pretherapy EUS or IDUS imaging showed concentric wall thickening of the distal common bile duct causing bile duct stenosis. ceEUS showed diffuse strong enhancement of the thickened bile duct wall, possibly due to inflammation. After the steroid therapy, the stenotic lesions in both the MPD and distal common bile duct were rapidly attenuated, with a decline in biochemical cholestatic enzymes and serum IgG or IgG4 levels.Conclusions On EUS and IDUS imaging, concentric bile duct wall thickening and its strong enhancement by Levovist was characteristic in AIP patients.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Intrasplenic pancreatic pseudocyst: a case report.

An intrasplenic pseudocyst associated with the acute relapsing phase of chronic pancreatitis in a 51-year-old woman is reported, with a review of the Japanese literature. The patient was admitted with a complaint of left lateral and back pain. Abdominal US and CT revealed communicating cysts at the pancreatic tail and the subcapsule of the spleen. A repeat US and CT 1 month after admission demonstrated enlargement of the cyst at the pancreatic tail. ERCP revealed a dilated main pancreatic duct without any definite evidence of stenosis, and direct communication with the cyst at the pancreatic tail. Percutaneous cystography revealed that the subcapsular cyst of the spleen, the cyst of the pancreatic tail, and the main pancreatic duct communicated with each other. The cyst contained serous fluid with an amylase content of 57,500 IU/I. Distal pancreatectomy and splenectomy was performed. Histologically, there was a nonepithelial lining on the inner surface of the cysts at the pancreatic tail and the subcapsule of the spleen. Severe chronic inflammatory changes were present in the resected tail of the pancreas. Timely surgical treatment is advocated to reduce the mortality and morbidity associated with complications of intrasplenic pseudocysts.     

Difficulty in diagnosing autoimmune pancreatitis by imaging findings

BACKGROUND: Autoimmune pancreatitis (AIP) presents as diffuse enlargement of the pancreas and as diffuse irregular narrowing of the main pancreatic duct. However, some AIP cases are difficult to diagnose because of atypical imaging findings. OBJECTIVE: To clarify a variety of imaging findings of AIP and the reason for its misdiagnosis. DESIGN: We examined the imaging findings of 37 AIP cases and also examined misdiagnosed cases of AIP to determine their reasons for misdiagnosis. PATIENTS: A total of 37 patients with AIP who reported to our hospital or its affiliate over a 17-year period (1989 to May 2005). RESULTS: Patients in 15 AIP cases showed segmental narrowing of the main pancreatic duct. There were 6 patients with focal enlargement of the pancreas, whereas 3 patients showed no enlargement. There were 3 cases of calcification of the pancreas. Pancreatic cysts were detected in 2 patients. Abdominal US showed multiple low-echoic masses in 1 case and a single mass in 3 cases. Sixteen patients had stenosis of the bile duct at the hilar hepatic lesion and/or the intrahepatic duct. Only 7 patients had typical AIP findings. Six patients were misdiagnosed with pancreatic cancer and 2 with bile-duct cancer. Seven cases were surgically treated. Five cases were misdiagnosed because of the nonexistence of, or the unfamiliarity with, the concept of AIP and sclerosing cholangitis with AIP. Another 3 cases were diagnosed with pancreatic cancer because of segmental stenosis of the main pancreatic duct and no or focal enlargement of the pancreas. CONCLUSIONS: The results of this study suggest that AIP presents a variety of imaging findings. The most important diagnostic factor is clinician awareness of the concept of AIP and the diverse nature of imaging findings.     

A Case of Groove Pancreatitis with Duodenal Stenosis

A 58‐year‐old man presented with a 2‐month history of nausea and vomiting. Blood levels of hepatic enzymes and pancreatitis markers were slightly elevated. Hypotonic duodenographic and endoscopic examinations revealed stenosis encircling the descending duodenum. A computed tomography (CT) scan showed inflammatory changes in the head of the pancreas and thickening of the duodenal wall. Magnetic resonance cholangiography demonstrated stenosis of the intrapancreatic segment of the common bile duct and diffuse dilatation of the main pancreatic duct, without irregularity. At laparotomy, microscopic examination of a needle biopsy specimen of the head of pancreas revealed no evidence of malignancy. Distal gastrectomy with Billroth II anastomosis was performed. Microscopically, fibrous thickening of the duodenal wall, serositis and hyperplasia of Brunner's glands were found. The presence of duodenal stenosis due to segmental pancreatitis, referred to as groove pancreatitis, was confirmed. The elevated blood levels of pancreatitis markers returned to the normal range 8 months after the operation. Ultrasonic echography and abdominal CT also revealed a marked reduction in dilatation of the extrahepatic bile duct and the main pancreatic duct. In patients suspected of having pancreatic carcinoma or regional pancreatitis, groove pancreatitis should be included in the differential diagnosis.     

Bile duct involvement in autoimmune pancreatitis: classification and treatment

Autoimmune pancreatitis is a rare disease characterized by inflammation of the pancreatic parenchyma, irregular narrowing of the pancreatic duct, periductal lymphoplasmacytic infiltration and fibrosis at histological examination, the presence of autoantibodies and hypergammaglobulinemia, as well as the possible association of cholangitis and other autoimmune diseases. There is a favorable response to steroid therapy. We report the case of a patient with autoimmune pancreatitis with bile duct involvement and peripheral eosinophilia, requiring long-term immunosuppressant treatment. The diagnosis of a diffuse form of AIP was made without direct histological evidence and based on indirect imaging, clinical and laboratory findings in an autoimmune context. The histological and imaging studies of bile duct involvement and the favourable response to steroids were additional arguments.     

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.