A case of IgG4-related disease presented with a duodenal ulcer to improve the understan-ding of IgG4-related diseases was reported. A 70-year-old male presented with cutaneous pruritus and abdominal pain for four years and blackened stools for two months. Four years ago, the patient went to hospital for cutaneous pruritus and abdominal pain. Serum IgG4 was 3.09 g/L (reference value 0-1.35 g/L), alanine aminotransferase 554 U/L (reference value 9-40 U/L), aspartate aminotransferase 288 U/L (reference value 5-40 U/L), total bilirubin 54.16 μmol/L (reference value 2-21 μmol/L), and direct bilirubin 29.64 μmol/L (reference value 1.7-8.1 μmol/L) were all elevated. The abdominal CT scan and magnetic resonance cholangiopancreatography indicated pancreatic swelling, common bile duct stenosis, and secondary obstructive dilation of the biliary system. The patient was diagnosed with IgG4-related disease and treated with prednisone at 40 mg daily. As jaundice and abdominal pain improved, prednisone was gradually reduced to medication discontinuation. Two months ago, the patient developed melena, whose blood routine test showed severe anemia, and gastrointestinal bleeding was diagnosed. The patient came to the emergency department of Beijing Hospital with no improvement after treatment in other hospitals. Gastroscopy revealed a 1.5 cm firm duodenal bulb ulcer. After treatment with omeprazole, the fecal occult blood was still positive. The PET-CT examination was performed, and it revealed no abnormality in the metabolic activity of the duodenal wall, and no neoplastic lesions were found. IgG4-related disease was considered, and the patient was admitted to the Department of Rheumatology and Immunology of Beijing Hospital for further diagnosis and treatment. The patient had a right submandibular gland mass resection history and diabetes mellitus. After the patient was admitted to the hospital, the blood test was reevaluated. The serum IgG4 was elevated at 5.44 g/L (reference value 0.03-2.01 g/L). Enhanced CT of the abdomen showed that the pancreas was mild swelling and was abnormally strengthened, with intrahepatic and extrahepatic bile duct dilation and soft tissue around the superior mesenteric vessels. We pathologically reevaluated and stained biopsy specimens of duodenal bulbs for IgG and IgG4. Immunohistochemical staining revealed remarkable infiltration of IgG4-positive plasma cells into duodenal tissue, the number of IgG4-positive cells was 20-30 cells per high-powered field, and the ratio of IgG4/IgG-positive plasma cells was more than 40%. The patient was treated with intravenous methylprednisolone at 40 mg daily dosage and cyclophosphamide, and then the duodenal ulcer was healed. IgG4 related disease is an immune-medicated rare disease characterized by chronic inflammation and fibrosis. It is a systemic disease that affects nearly every anatomic site of the body, usually involving multiple organs and diverse clinical manifestations. The digestive system manifestations of IgG4-related disease are mostly acute pancreatitis and cholangitis and rarely manifest as gastrointestinal ulcers. This case confirms that IgG4-related disease can present as a duodenal ulcer and is one of the rare causes of duodenal ulcers.
Aged ; Humans ; Male ; Abdominal Pain/drug therapy* ; Acute Disease ; Bilirubin ; Duodenal Ulcer/etiology* ; Immunoglobulin G ; Immunoglobulin G4-Related Disease/diagnosis* ; Pancreatitis/drug therapy* ; Positron Emission Tomography Computed Tomography ; Prednisone/therapeutic use* ; Pruritus/drug therapy*

Hypertriglyceridemia a major cause of acute pancreatitis, accounting for up to 10% of all cases. The pathophysiological mechanism of hypertriglyceridemia-induced acute pancreatitis (HTGP) is presumed to involve the hydrolysis of triglycerides by pancreatic lipase resulting in an excess of free fatty acids and elevated chylomicrons, which are thought to increase plasma viscosity and induce ischemia and inflammation in pancreatic tissue. Although the clinical course of HTGP is similar to other forms of acute pancreatitis, the clinical severity and associated complications are significantly higher in patients with HTGP. Therefore, an accurate diagnosis is essential for treatment and prevention of disease recurrence. At present, there are no approved guidelines for the management of HTGP. Different treatment modalities such as apheresis/plasmapheresis, insulin, heparin, fibric acids, and omega-3 fatty acids have been successfully implemented to reduce serum triglycerides. Following acute phase management, lifestyle modifications including dietary adjustments and drug therapy are important for the long-term management of HTGP and the prevention of relapse. Additional studies are required to produce generalized and efficient treatment guidelines for HTGP.
Chylomicrons ; Diagnosis ; Drug Therapy ; Fatty Acids, Nonesterified ; Fatty Acids, Omega-3 ; Fibric Acids ; Heparin ; Humans ; Hydrolysis ; Hypertriglyceridemia ; Inflammation ; Insulin ; Ischemia ; Life Style ; Lipase ; Pancreatitis* ; Plasma ; Recurrence ; Triglycerides ; Viscosity

Acute Disease ; Humans ; Male ; Middle Aged ; Pancreatitis, Chronic ; diagnosis ; drug therapy ; Purpura, Schoenlein-Henoch ; diagnosis ; drug therapy ; Steroids ; therapeutic use ; Tomography, X-Ray Computed

No abstract available.
Acute Disease ; Biopsy ; Fatal Outcome ; Female ; Fluorescent Antibody Technique ; Glomerulonephritis/*complications/diagnosis/drug therapy/immunology ; Humans ; Immunosuppressive Agents/therapeutic use ; Microscopic Polyangiitis/*complications/diagnosis/drug therapy/immunology ; Middle Aged ; Pancreatitis/diagnosis/drug therapy/*etiology/immunology ; Treatment Outcome

Autoimmune pancreatitis (AIP),a special type of chronic pancreatitis,is autoimmune-mediated and can be accompanied by swelling of the pancreas and irregular stenosis of the pancreatic duct. The main pathological features are fibrosis of pancreatic duct with IgG4-positive lymphoplasmacytic infiltration. Different typing methods of AIP can have differerent disease conditions. This paper reviews the history,clinical presentation,diagnostic criteria,and treatment of different AIP types to provide a new basis for the diagnosis and treatment.
Autoimmune Diseases ; diagnosis ; therapy ; Fibrosis ; Humans ; Immunoglobulin G ; blood ; Pancreas ; physiopathology ; Pancreatitis ; diagnosis ; therapy

OBJECTIVETo investigate the clinical feature, diagnostic and therapeutic experience of autoimmune pancreatitis (AIP).

METHODSTwenty-one patients with AIP treated in the First Affiliated Hospital, Harbin Medical University from January 2006 to July 2014 were analyzed retrospectively. There were 15 men and 6 women among the 21 cases and the age ranged from 36 to 64 years. The characters of diagnosis and treatment of AIP were explored through clinical symptoms, imaging features, serologic test results, diagnostic treatment, and histopathologic characteristics.

RESULTSAll the patients showed obstructive jaundice and upper abdominal pain to different extents as major manifestations and the levels of serum IgG4, CA19-9, CEA were elevated in 16 cases (76.2%), 6 cases (28.5%) and 3 cases (14.2%), respectively. CT showed diffuse enlargement of the pancreas in 9 cases, localized pancreatic head enlargement in 3 cases and focally pancreatic mass in 9 cases. AIP was confirmed by extrapancreatic involvement, radiological and serological results plus biopsy in 11 cases (52.4%), interpretation of response to steroid in 3 cases (14.3%) and open laparotomy in 7 cases (33.3%). Surgery included choledochojejunostomy in 3 cases, cholecystojejunostomy in 1 case, pancreaticoduodenectomy in 2 cases and distal pancreatectomy combined with splenectomy in 1 case. The pathologic results displayed massive lymphocytes and plasma cells infiltration in the pancreatic tissues as well as parenchymal fibrosis. Except for 1 patient who had no symptom, the regular steroid therapy was performed (oral prednisone) and all the patients were cured. The follow-up time range was from 3 to 93 months, 4 cases (19.0%) were recurrent followed by the symptoms alleviated after the steroid was applied again.

CONCLUSIONSAIP is rare and characterized by non-specific clinical manifestations so that the early diagnosis is difficult with a high misdiagnosis rate. The clinicians should strengthen the recognition of AIP and the definite diagnosis depends on the combination of clinical manifestations, radiological, serological and histopathological results so as to avoid the unnecessary operation.

Adult ; Autoimmune Diseases ; diagnosis ; therapy ; Biopsy ; CA-19-9 Antigen ; blood ; Carcinoembryonic Antigen ; blood ; Diagnostic Imaging ; Female ; Humans ; Immunoglobulin G ; blood ; Male ; Middle Aged ; Pancreas ; pathology ; Pancreatectomy ; Pancreatitis ; diagnosis ; therapy ; Retrospective Studies ; Steroids ; therapeutic use

The diagnostic and therapeutic course of acute pancreatitis is complicated. The treatment strategy existed dispute. The management of acute pancreatitis includes intensive care, fluid resuscitation, organ function support, infection control, endoscopic interventional management, surgical operation and other fields. The diagnosis and treatment mode of multidisciplinary integrate collaboration has been established. It will make the greatest benefit if we depend on evidence-based medicine, play the superiority of professional team and make individual treatment protocols.
Acute Disease ; Critical Care ; Evidence-Based Medicine ; Fluid Therapy ; Humans ; Pancreatitis ; diagnosis ; therapy

BACKGROUND/AIMS: Acute pancreatitis is a common complication of endoscopic retrograde cholangiopancreatography (ERCP). Combination therapy w ith ora l udenafil and aceclofenac may reduce the occurrence of post-ERCP pancreatitis by targeting different pathophysiological mechanisms. We investigated whether combining udenafil and aceclofenac reduced the rates of post-ERCP pancreatitis. METHODS: A prospective, randomized, double-blind, placebo-controlled, multicenter study was conducted in four academic medical centers. Between January 2012 and June 2013, a total of 216 patients who underwent ERCP were analyzed for the occurrence of post-ERCP pancreatitis. Patients were determined to be at high risk for pancreatitis based on validated patient and procedure-related risk factors. RESULTS: Demographic features, indications for ERCP, and therapeutic procedures were similar in each group. There were no significant differences in the rate (15.8% [17/107] vs. 16.5% [18/109], p = 0.901) and severity of post-ERCP pancreatitis between the udenafil/aceclofenac and placebo groups. One patient in each group developed severe pancreatitis. Multivariate analyses indicated that suspected dysfunction of the sphincter of Oddi and endoscopic papillary balloon dilation without sphincterotomy were associated with post-ERCP pancreatitis. CONCLUSIONS: Combination therapy with udenafil and aceclofenac is not effective for the prevention of post-ERCP pancreatitis.
Acute Disease ; Administration, Oral ; Adult ; Aged ; Aged, 80 and over ; Anti-Inflammatory Agents, Non-Steroidal/*administration & dosage/adverse effects ; Cholangiopancreatography, Endoscopic Retrograde/*adverse effects ; Diclofenac/administration & dosage/adverse effects/*analogs & derivatives ; Double-Blind Method ; Drug Therapy, Combination ; Female ; Humans ; Male ; Middle Aged ; Multivariate Analysis ; Pancreatitis/diagnosis/etiology/*prevention & control ; Phosphodiesterase 5 Inhibitors/*administration & dosage/adverse effects ; Prospective Studies ; Pyrimidines/*administration & dosage/adverse effects ; Republic of Korea ; Risk Factors ; Sulfonamides/*administration & dosage/adverse effects ; Treatment Outcome ; Young Adult

Portal vein thrombosis is an uncommon but an important cause of portal hypertension. The most common etiological factors of portal vein thrombosis are liver cirrhosis and malignancy. Albeit rare, portal vein thrombosis can also occur in the presence of local infection and inflammation such as pancreatitis or cholecystitis. A 52-year-old male was admitted because of general weakness and poor oral intake. He had an operation for colon cancer 18 months ago. However, colonic stent had to be inserted afterwards because stricture developed at anastomosis site. Computed tomography taken at admission revealed portal vein thrombosis and inflammation at colonic stent insertion site. Blood culture was positive for Escherichia coli. After antibiotic therapy, portal vein thrombosis resolved. Herein, we report a case of portal vein thrombosis with sepsis caused by inflammation at colonic stent insertion site which was successfully treated with antibiotics.
Anti-Bacterial Agents/therapeutic use ; Cholecystitis/etiology ; Colonic Neoplasms/pathology/therapy ; Escherichia coli/isolation & purification ; Escherichia coli Infections/drug therapy/etiology ; Humans ; Inflammation/*etiology ; Liver/diagnostic imaging ; Male ; Middle Aged ; Pancreatitis/etiology ; Portal Vein ; Sepsis/*diagnosis/drug therapy/microbiology ; Sigmoidoscopy ; Stents/*adverse effects ; Tomography, X-Ray Computed ; Venous Thrombosis/complications/*diagnosis

Accurate diagnosis of autoimmune pancreatitis (AIP) is important to clinicians since it is difficult to differentiate AIP from pancreatic malignancies. Furthermore, unlike pancreatic malignancies, AIP has dramatic response to steroids. A 61-years-old man presented with acute pancreatitis. Imaging studies showed two separate pancreatic masses, irregular narrowing of main pancreatic duct, and a renal mass that highly suggested AIP. Endoscopic ultrasound-guided core needle biopsy of the pancreatic masses and ultrasound-guided biopsy of the renal mass revealed peripheral T-cell lymphoma. The patient is currently undergoing chemotherapy. We present a case of pancreatic lymphoma masquerading as AIP with literature review.
Biopsy ; Biopsy, Large-Core Needle ; Diagnosis ; Drug Therapy ; Humans ; Lymphoma* ; Lymphoma, T-Cell, Peripheral ; Pancreatic Ducts ; Pancreatic Neoplasms ; Pancreatitis* ; Steroids