Autoimmune pancreatitis associated with a large pancreatic pseudocyst that disappeared after corticosteroid therapy: a case report and literature review

A 51-year-old woman was admitted to our department because of upper abdominal pain. The serum IgG4 concentration was elevated, and abdominal computed tomography revealed diffuse enlargement of the pancreas associated with a large cyst, measuring 8 cm in diameter. Endoscopic retrograde cholangiopancreatography revealed narrowing of the main pancreatic duct (from the body to the tail), narrowing of the intrapancreatic bile duct, and dilatation of the bile ducts. The patient was given a diagnosis of autoimmune pancreatitis (AIP) associated with a pancreatic pseudocyst and intrapancreatic bile duct stenosis. Oral steroid therapy resulted in reduced pancreatic swelling, complete disappearance of the pancreatic cyst, and an improvement in biliary stenosis. AIP is rarely associated with pancreatic cyst, and only 13 cases, including ours, have been reported to date. In our patient, intense inflammation apparently led to cyst formation in association with AIP, which responded remarkably to corticosteroid therapy. Correct diagnosis of AIP associated with a pancreatic pseudocyst might save patients from undergoing unnecessary endoscopic and surgical procedures.     

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…     

Uncomplicated spontaneous rupture of pancreatic pseudocyst into stomach: A case report

Pseudocysts of the pancreas are not rare, but spontaneous perforation and/or fistulization occurs in fewer than 3% of these pseudocysts. Perforation into the free peritoneal cavity, stomach, duodenum, colon, portal vein, pleural cavity and through the abdominal wall has been reported. Spontaneous rupture of the pancreatic pseudocyst into the surrounding hollow viscera is rare and, may be associated with life-threatening bleeding. Such cases require emergency surgical intervention. Uncomplicated rupture of pseudocyst is an even rarer occurrence. We present a case of spontaneous resolution of a pancreatic pseudocyst with gastric connection without bleeding. A 67-year-old women with a large pancreatic pseudocyst resulting from a complication of chronic pancreatitis was referred to our institution. During hospital stay, there was sudden decrease in the size of epigastric lump. Repeat computed tomography(CT) revealed that the size of the pseudocyst had decreased significantly; however, gas was observed in stomach and pseudocyst along with rent between lesser curvature of stomach and pseudocyst suggestive of spontaneous cystogastric fistula.The fistula tract occluded spontaneously and the patient recovered without any complication or need for surgical treatment. After 5 wk, follow up CT revealed complete resolution of pseudocyst. Esophagogastroduodenoscopy revealed that the orifice was completely occluded with ulcer at the site of previous fistulous opening.     

Intramedullary fat necrosis, polyarthritis and panniculitis with pancreatic tumor: a case report

Acute polyarthritis can occur in non-rheumatic systemic illnesses, presenting a diagnostic dilemma. We present an extremely rare case presenting as acute polyarthritis, panniculitis and medullary fat necrosis with underlying pancreatic pathology. This case report describes a young woman presenting with panniculits, pancreatic tumour, polyarthritis and intra-osseus fat necrosis with a fatal outcome. The medical fraternity needs to be aware of this potentially fatal albeit rare musculoskeletal complication secondary to a pancreatic pathology.     

Childhood pancreatic trauma: Clinical presentation,natural history and outcome

ObjectivesTo study the presentation, management strategies and long-term natural history of children with pancreatic trauma.MethodsChildren admitted with pancreatic trauma were analyzed for their presentation, management and outcome. Management included nasojejunal feeds, total parenteral nutrition (TPN), octreotide, drainage (radiological and endoscopic), endoscopic retrograde cholangiopancreatography (ERCP) and surgery. Patients were assessed in follow-up for development of chronic pancreatitis (CP).Results36 children [29 boys, age 144 (13–194) months] presented at 30 (3–210) days after trauma. Most common cause of trauma was bicycle handle bar injury [n = 18,50%]. Presenting features were abdominal pain [n = 26,72%], lump [n = 16, 44.4%], ascites [n = 13,36%], pleural effusion [n = 9,25%] and anasarca [n = 3,8.3%]. All presented with sequelae of ductal disruption with pseudocyst, ascites or pleural effusion. Fifteen (41.6%) patients each had Grade III and IV injury, 4 (11%) had grade V, and grading was unavailable in 2. Other organs were injured in 4 (11%) cases. Management consisted of various combinations of nasojejunal feeds [n = 17,47.2%], TPN [n = 5,13.8%], octreotide [n = 13,36%], pseudocyst drainage [radiological (n = 18,50%), endoscopic (n = 3,8.3%)] and ERCP [n = 12,33.3%]. Surgical intervention was done in 2 (5.5%) cases [cystojejunostomy and peritoneal lavage in 1 each]. Two (5.5%) patients died due to sepsis. Of the 32 cases in follow-up, 19 (59.3%) recovered and 13 (40.6%) developed CP, with half (6/13) of them being symptomatic with recurrent pain.ConclusionMulti-disciplinary non-operative management is effective for managing pancreatic trauma in 94.4% of children, with 75% requiring radiological or endoscopic intervention. 40% developed structural changes later but only half were symptomatic.     

慢性胰腺炎的并发症

目的：探讨慢性胰腺炎（Chronic pancreatitis,CP）并发症的发生率、临床表现及诊断,旨在提高对于CP并发症的认识。方法：回顾性分析北京协和医院1982年－2001年确诊CP的236例住院患者。所有病例均符合1983年日本胰腺病协会关于CP的诊断标准,其中经病理证实者82例。结果：236例CP患者中156例出现并发症,占66．1％。其中,胆道梗阻、狭窄41例,占236例CP的17．4％;胰腺假性囊肿40例,占16．9％,脾静脉狭窄32例,占13．6％;胰源性浆膜腔积液19例,占8．1％; 上消化道不全梗阻17例,占7．2％;消化性溃疡14例,占5．9％;邻近血管病变13例,占5．5％;合并症还涉及胰腺癌（占4．2％）、结核及胰外肿瘤。结论：CP并发症相当常见,临床表现丰富多彩,有些患者以 并发症首诊,应提高对CP并发症的认识。     

Patient reported exposure to smoking and alcohol abuse are associated with pain and other complications in patients with chronic pancreatitis

Background/objectivesSmoking and alcohol abuse are established risk factors for chronic pancreatitis (CP). Few studies have examined how exposure to smoking and alcohol abuse act as risk factors for complications in CP. Our aim was to examine associations between patient reported exposure to smoking and alcohol abuse and complications in CP in a large cohort of patients from the Scandinavian and Baltic countries.MethodsWe retrieved data on demographics, CP related complications and patients’ histories of exposure to smoking and alcohol abuse from the Scandinavian Baltic Pancreatic Club database. Associations were investigated by univariate and multivariate logistic regression analyses. Results are presented as odds ratios (OR) with 95% confidence intervals.ResultsA complete history of smoking and alcohol exposure was available for 932 patients. In multivariate regression analyses, the presence of pain and exocrine pancreatic insufficiency were both significantly associated with history of smoking (OR 1.94 (1.40–2.68), p < 0.001 and OR 1.89 (1.36–2.62), p < 0.001, respectively) and alcohol abuse (OR 1.66 (1.21–2.26), p = 0.001 and 1.55 (1.14–2.11), p = 0.005, respectively). Smoking was associated with calcifications (OR 2.89 (2.09–3.96), p < 0.001), moderate to severe ductal changes (OR 1.42 (1.05–1.92), p = 0.02), and underweight (OR 4.73 (2.23–10.02), p < 0.001). History of alcohol abuse was associated with pseudocysts (OR 1.38 (1.00–1.90) p = 0.05) and diabetes mellitus (OR 1.44 (1.03–2.01), p = 0.03). There were significantly increased odds-ratios for several complications with increasing exposure to smoking and alcohol abuse.ConclusionSmoking and alcohol abuse are both independently associated with development of complications in patients with CP. There seems to be a dose-dependent relationship between smoking and alcohol abuse and complications in CP.     

A Case of Autoimmune Pancreatitis Manifested by a Pseudocyst and IgG4-Associated Cholangitis

Autoimmune pancreatitis (AIP) is a benign disorder and a unique form of chronic pancreatitis with several characteristic features. A cystic formation that mimics a pseudocyst is a rare finding. There have been a few reports of AIP complicated by pancreatic cysts. We present a case of AIP with multiple pseudocysts and obstructive jaundice caused by IgG4-associated cholangitis. We initially missed the diagnosis due to the pseudocyst. Based on the computed tomography images, laboratory findings and the therapeutic response to steroids, the case was diagnosed as AIP with pseudocysts and associated cholangiopathy.     

重症急性胰腺炎的并发症及治疗体会

目的：总结重症急性胰腺炎的主要并发症及治疗体会。方法：回顾性分析１９９４年６月至１９９８年１２月收治的重症急性胰腺炎３９例,对主要并发症的原因及治疗方法进行总结。结果：３９例重症急性胰腺炎主要并发症为肺损害、休克、腹腔感染、多器官功能紊乱等,治愈率达７２％。结论：重症急性胰腺炎并发症多,应重点防治ＡＲＤＳ、休克、腹腔感染等。     

Difficulty with diagnosis of malignant pancreatic neoplasms coexisting with chronic pancreatitis

Chronic pancreatitis is a relatively common disease. We encountered two different cases of belatedly demonstrated pancreatic carcinoma featuring underlying chronic pancreatitis. The first case was one that was highly suspected as that of a malignancy based upon imaging study, but unfortunately, it could not be confirmed by intra-operative cytology at that time. Following this, the surgeon elected to perform only conservative bypass surgery for obstructive biliary complication. Peritoneal carcinomatosis was later noted and the patient finally died. The second case, a malignant mucinous neoplasm, was falsely diagnosed as a pseudocyst, based upon the lesion's sonographic appearance and associated elevated serum amylase levels. After suffering repeated hemoptysis, the patient was found to exhibit lung metastasis and peritoneal seeding. We reviewed some of the literature, including those studies discussing chronic pancreatitis predisposing to a malignant change. These two case analyses illustrate clearly that the diagnosis for such conditions, which is simply based upon imagery or pathological considerations may end up being one of a mistaken malignancy. Some of our suggestions for the treatment of such malignancies as revealed herein include, total pancreatomy for univocal mass lesion, and needle aspiration of lesion-contained tissue for amylase, CA199 and CEA levels for a suspicious cystic pancreatic mass.     

Management of chronic pancreatitis complicated with a bleeding pseudoaneurysm

Chronic pancreatitis is an ongoing disease characterized by persistent inflammation of pancreatic tissues.With disease progression,patients with chronic pancreatitis may develop troublesome complications in addition to exocrine and endocrine pancreatic functional loss.Among them,a pseudoaneurysm,mainly induced by digestive enzyme erosion of vessels in proximity to the pancreas,is a rare and life-threatening complication if bleeding of the pseudoaneurysm occurs.At present,no prospective randomized trials have investigated the therapeutic strategy for this rare but critical situation.The role of arterial embolization,the timing of surgical intervention and even surgical procedures are still controversial.In this review,we suggest that dynamic abdominal computed tomography and angiography should be performed first to localize the bleedersand to evaluate the associated complications such as pseudocyst formation,followed by arterial embolization to stop the bleeding and to achieve early stabilization of the patient’s condition.With advances and improvements in endoscopic devices and techniques,therapeutic endoscopy for pancreatic pseudocysts is technically feasible,safe and effective.Surgical intervention is recommended for a bleeding pseudoaneurysm in patients with chronic pancreatitis who are in an unstable condition,for those in whom arterial embolization of the bleeding pseudoaneurysm fails,and when endoscopic management of the pseudocyst is unsuccessful.If a bleeding pseudoaneurysm is located over the tail of the pancreas,resection is a preferential procedure,whereas if the lesion is situated over the head or body of the pancreas,relatively conservative surgical procedures are recommended.     

Necrotizing pancreatitis complicated by oesophageal haemorrhage

Bleeding is a rare complication of pancreatic pseudocyst. We describe an exceptional case of necrotizing pseudocyst with mediastinal extension providing cataclysmic oesophageal haemorrhage. The patient was successfully treated by adequate endoscopic, radiological and surgical management.     

Role of Osteopontin in Calcification in Autoimmune Pancreatitis

Objectives The aim of the present study was to determine the potential for pancreatic calcification in autoimmune pancreatitis by investigating osteopontin and CD44 expression. Methods Human pancreatic tissues in normal pancreas, chronic pancreatitis, and autoimmune pancreatitis were obtained from the surgical specimens of 42 patients. Pancreatic tissues from male Wistar Bonn/Kobori rats were also used as an animal autoimmune pancreatitis model. Results The incidences of osteopontin expression in centroacinar cells in chronic pancreatitis with calcification and in autoimmune pancreatitis were significantly greater than that in normal pancreas (P < 0.05). Some cases of chronic pancreatitis and autoimmune pancreatitis expressed CD44 in centroacinar cells and ductal cells. In male Wistar Bonn/Kobori rats, the inflammatory area and percentage of osteopontin-CD44-positive cells increased with advancing age (P < 0.01 or 0.05). Conclusions These results suggest that autoimmune pancreatitis has the potential for pancreatic calcification over a long-term clinical course.     

‘Pancreatitis, polyarthritis, panniculitis syndrome’ (PPP syndrome) plus prolonged pyrexia-a rare presentation of chronic pancreatitis

Pancreatitis presenting without abdominal pain is very unusual. Here we report a 70-year-old man with chronic calcific pancreatitis presented to us with prolonged fever, arthritis and multiple subcutaneous swellings, but without any abdominal pain or other abdominal symptoms. His serum amylase and lipase were very high. Biopsy from the subcutaneous swellings revealed fat necrosis and CT scan abdomen showed features of chronic calcific pancreatitis. He was managed conservatively with supportive measures, and recovered. There is only scanty information in literature regarding this type of presentation in chronic pancreatitis.     

Morphological and Functional Evaluation of the Pancreatic Duct with Secretin-Stimulated Magnetic Resonance Cholangiopancreatography in Alcoholic Pancreatitis Patients

Objectives The aim of this investigation was to evaluate the pancreatographic findings and dynamics of pancreatic duct diameter, as determined by secretin-enhanced magnetic resonance cholangiopancreatography (S-MRCP), in patients with acute alcoholic pancreatitis or chronic alcoholic pancreatitis and in a control group. Methods S-MRCP was performed in patients with acute alcoholic pancreatitis who did not manifest the functional and radiological (ultrasonography and computed tomography) criteria of chronic pancreatitis (n = 21), in patients with chronic alcoholic pancreatitis (n = 28) and in a control group (n = 16). The diameter of the main pancreatic duct (MPD) was monitored before secretin administration and at 3 and 10 min after secretin administration. Morphological features were also assessed before and after the administration of secretin. Results All ductal diameters were significantly larger in chronic alcoholic pancreatitis (P < 0.0001). There were no differences in MPD caliber between patients with acute alcoholic pancreatitis and the control group. The percentage of variation between basal MPD diameter and at 3 min post-secretin administration was lower in patients with chronic (35.5%) pancreatitis than in those with acute alcoholic pancreatitis (52.3%) and the control group (52.5%). There were no significant differences between patients with acute alcoholic pancreatitis and the control group in terms of the frequency of visualization of side branches, ductal narrowing, intraluminal filling defects, and ductal irregularity. One patient with acute alcoholic pancreatitis presented ductal criteria of chronic pancreatitis following the administration of secretin. Conclusions The dynamics of MPD visualized on S-MRCP in patients with acute alcoholic pancreatitis is similar to that observed in the control group and different from that observed in patients with chronic alcoholic pancreatitis. There were no significant differences between patients with acute alcoholic pancreatitis and the control group in terms of morphological pancreatographic features.     

慢性胰腺炎的相关因素及诊治分析

目的研究我国慢性胰腺炎（CP）的相关因素及诊治特点。方法回顾分析我国近10年21所综合医院确诊为CP的1700例住院患者，调查其相关因素、诊断方法及治疗措施。结果近年我国CP的发病有增多趋势，男性多于女性。相关因素中以慢性乙醇中毒为主，1700例患者中，酒精性601例（35．4％），胆源性576例（33．9％），其他相关因素包括自身免疫性疾病、胰腺外伤等。反复发作的腹痛为最常见症状，少数患者表现为脂肪泻或体重减轻等。组织学检查是CP诊断的金标准，但大部分患者经由影像学检查及胰腺外分泌试验（BTPABA试验）诊断。85.9％的患者经非手术治疗获得症状缓解。结论重度饮酒已成为我国CP的主要致病因素，影像学检查在CP诊断中具有重要作用，非手术治疗是目前治疗慢性胰腺炎的主要方法。     

Acute Myeloid Leukemia Developing with Acute Pancreatitis Mimicking Autoimmune Pancreatitis

A 33-year-old man was admitted to our hospital for fever and abdominal pain. A blood analysis revealed pancytopenia and increased serum pancreatic enzymes with disseminated intravascular coagulation. A detailed examination revealed acute pancreatitis, with diffuse swelling of the pancreas and diffuse beaded dilatation of the main pancreatic duct, which mimicked autoimmune pancreatitis complicated by acute myeloid leukemia. Systemic cytotoxic chemotherapy led to the remission of leukemia and pancreatitis. We hypothesized that the etiology of acute pancreatitis was invasion of leukemia cells. Acute pancreatitis is rare as a symptom of leukemia; however, we should consider the possibility of leukemia during the differential diagnosis of acute pancreatitis.