No abstract availble.
Autoimmune Diseases/*diagnosis ; Bile Duct Diseases/*diagnosis ; Cholangitis, Sclerosing/*diagnosis ; Diagnosis, Differential ; Female ; Humans ; Male ; Pancreatitis/*diagnosis/immunology

Immunoglobulin G4 (IgG4)-related sclerosing disease is a systemic disease characterized by extensive IgG4-positive plasma cells and T-lymphocyte infiltration in various organs. We described the imaging findings of an IgG4-related inflammatory pseudotumor in the urethra. The urethral mass showed isoattenuation on unenhanced CT images, delayed enhancement on enhanced CT images, iso- to slight hyper-intensity on T1 and T2 weighted magnetic resonance images, diffusion restriction on diffusion weighted images, and heterogeneously low echogeneity on ultrasonography.
Aged ; Autoimmune Diseases/diagnosis ; Female ; Granuloma, Plasma Cell/*diagnosis ; Humans ; Immunoglobulin G/*immunology ; Pancreatitis/diagnosis/immunology ; Sclerosis ; Urethral Diseases/*diagnosis/immunology

BACKGROUND/AIMS: To diagnose autoimmune pancreatitis (AIP), serum IgG and IgG4 concentration are significant serologic markers. The purpose of this study was to assess the utility of serum IgG and IgG4 for the diagnosis of AIP. METHODS: We divided enrolled patients into 2 groups: autoimmune pancreatitis and other pancreatic disease. We measured serum IgG and IgG4 levels in patients including 12 AIP and 23 other pancreatic disease. RESULTS: Among AIP patients, 10 cases (83%) showed elevated IgG4 levels of more than 135 mg/dL and 4 cases (33%) showed elevated IgG levels of more than 1,800 mg/dL, the current cutoff value applied in Japan. Only one patient showed elevated serum IgG level, despite having normal IgG4 level. Sensitivity and specificity for AIP of elevated serum IgG4 (>135 mg/dL) were 91% and 92%, and for elevated serum IgG (>1,800 mg/dL) 67% and 92%, respectively. The optimal cut-off level of IgG4 using receiver operation characteristic (ROC) was 127 mg/dL, at which the sensitivity and specificity were 83% and 96%, respectively, for the diagnosis of AIP. Serum IgG at 1,520 mg/dL showed the sensitivity and specificity of 83% and 87%, respectively. CONCLUSONS: The measurement of serum IgG and IgG4 are helpful to diagnose AIP. Serum IgG and IgG4 are complementary to each other in the diagnosis of AIP.
Adult ; Aged ; Autoimmune Diseases/*diagnosis/immunology ; Biological Markers/blood ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulin G/*blood ; Male ; Middle Aged ; Pancreatitis, Chronic/*diagnosis/etiology/immunology ; ROC Curve ; Sensitivity and Specificity

BACKGROUND/AIMS: Autoimmune pancreatitis is a distinctive type of chronic pancreatitis with reversibility. We analyzed the largest number of patients with autoimmune pancreatitis among the studies carried out at a single institution in Korea. METHODS: We retrospectively analyzed the clinical, radiologic, laboratory and histologic features in 17 patients who were diagnosed as autoimmune pancreatitis. RESULTS: The patients were predominately elderly men who presented with jaundice or nonspecific gastrointestinal symptoms. In most of the patients, serum pancreatic enzymes were normal or mildly elevated with an accompanying cholestatic biochemical profile. Elevated IgG levels and detection of autoantibodies were observed in 47% (8/17) and 35% (6/17), respectively. Thirteen patients showed histologic findings of lymphoplasmacytic infiltration and fibrosis, and one patient showed predominant infiltration of eosinophils. Fifteen patients revealed radiologic images of diffuse swelling of pancreas and the sclerosing pattern on direct pancreatogram. All of the features improved in response to oral steroid therapy. Two cases showed focal narrowing with upstream duct dilatation and thus, they were diagnosed as focal type. Diabetes mellitus concurred in 13 cases and primary sclerosing cholangitis and Sjogren's syndrome were present in 1 case, respectively. CONCLUSIONS: The recognition of autoimmune pancreatitis allows patients to avoid unnecessary surgery and recover with the administration of oral steroid.
Aged ; Autoimmune Diseases/*diagnosis/pathology ; Chronic Disease ; English Abstract ; Humans ; Male ; Middle Aged ; Pancreas/pathology ; Pancreatitis/*diagnosis/immunology/pathology

Systemic amyloidosis is a disorder characterized by extracellular deposition of amyloid in various organs and tissues including the kidney, heart, and liver. However, pancreatic involvement is rare, and has not been reported in Korea. Systemic amyloisosis involving pancreas needs to be differentiated from several pancreatic diseases because of diffuse pancreatic enlargement and partial stricture or obstruction of main pancreatic duct. Recently, we experienced a 60-year old man who was suspected as autoimmune pancreatitis or infiltrative disorders on imaging studies, and finally diagnosed as systemic amyloidosis involving pancreas and liver on biopsy examination. We report the case with review of the relevant literatures.
Amyloidosis/*diagnosis/pathology/radiography ; Autoimmune Diseases/diagnosis ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis, Differential ; Humans ; Male ; Middle Aged ; Pancreatic Diseases/*diagnosis ; Pancreatitis/diagnosis/immunology/pathology ; Tomography, X-Ray Computed

Autoimmune Diseases ; diagnosis ; immunology ; pathology ; surgery ; Humans ; Immunoglobulin G ; blood ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pancreatectomy ; Pancreatitis ; diagnosis ; immunology ; pathology ; surgery

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

No abstract available.
Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/*diagnosis/drug therapy ; Humans ; Immunoglobulin G/blood ; Male ; Middle Aged ; Pancreatitis/complications/*diagnosis/immunology ; Prednisolone/therapeutic use ; Retroperitoneal Fibrosis/complications/*diagnosis/immunology ; Tomography, X-Ray Computed

No abstract available.
Autoimmune Diseases/*diagnosis/drug therapy/immunology ; Azathioprine/therapeutic use ; Common Bile Duct/pathology ; Emphysema ; Fibrosis ; Humans ; Immunoglobulin G/blood ; Immunosuppressive Agents/therapeutic use ; Lung/*radiography ; Male ; Middle Aged ; Pancreatitis/*diagnosis/drug therapy/immunology ; Stents ; Tomography, X-Ray Computed

OBJECTIVETo explore the clinical, pathological, and imaging features of autoimmune pancreatitis (AlP).

METHODSThe clinical data of 10 patients (all men; aged 47-80 years, mean 61.3 years) with AlP in our hospital between March 2000 and August 2007 were retrospectively analyzed. gamma-globulin, immunoglobulin C (IgG), rheumatoid factors, and autoantibodies were examined for all cases. The imaging findings were reviewed, which included helical computed tomography (CT), endoscopic ultrasonography (EUS), and B-mode ultrasound in all patients, magnetic resonance cholangiopancreatography (MRCP) in 9 patients, and endoscopic retrograde cholangiopancreatography (ERCP) in 7 patients. Follow-up imaging results were available in 5 patients.

RESULTSClinically, the most common early symptoms included obstructive jaundice (9/10) and non-specific abdominal pain (1/10), accompanied by the elevated levels of serum gamma-globulin, IgG or the presence of autoantibodies. Diabetes mellitus was detected at presentation in 2 patients. imaging findings included: CT showed diffuse (n=9) and focal (n=1) enlargement of pancreas. Minimal peripancreatic stranding was found in 7 patients, with no pancreatic pseudocyst and calcification. Six patients had enlarged peripancreatic lymph nodes. After contrast injection for 4 patients, delayed enhancement of the pancreatic parenchyma was observed, along with low-density capsule-like rim surrounding the pancreas. Magnetic resonance imaging showed diffuse enlargement of pancreas in 9 patients. MRCP showed diffuse (n=6) and segmental (n=3) irregular narrowing of the main pancreatic duct. B-mode ultrasound showed diffuse (n=9) and focal (n=1) enlargement of the pancreas. EUS showed diffuse (n=9) and focal (n=1) enlargement with hypoecho. ERCP showed stricture of distal common bile duct and irregular dilation of proximal bile ducts in 7 patients, diffuse stricture in main pancreatic duct in 4 patients, and segmental stricture in 3 patients. During the follow-up, abnormalities of imaging and serum markers were resolved after steroid therapy in 5 patients.

CONCLUSIONAIP is a distinctive type of chronic pancreatitis that shows specific imaging features.

Aged ; Aged, 80 and over ; Autoantibodies ; blood ; Autoimmune Diseases ; diagnosis ; diagnostic imaging ; immunology ; Endosonography ; Female ; Humans ; Male ; Middle Aged ; Pancreatitis ; diagnosis ; diagnostic imaging ; immunology ; Retrospective Studies ; Tomography, X-Ray Computed