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

Korean autoimmune pancreatitis (AIP) criteria 2007 was aimed to diagnose the wide spectrum of AIP with high sensitivity. The most crucial issue when caring for patients with suspected AIP is to differentiate AIP from pancreatic cancer. Pancreatic cancer can be distinguished from AIP by pancreatic imaging, measurement of serum IgG4 levels, endoscopic ultrasound guided fine needle aspiration and trucut biopsy, and steroid trial. Autoimmune pancreatitis is a rare systemic fibroinflammatory disease which can affect not only the pancreas, but also a variety of organs such as the bile ducts, salivary glands, retroperitoneum, and lymph nodes. Organs affected by AIP have a lymphoplasmacytic infiltrate rich in IgG4-positive cells. This inflammatory process responds dramatically to oral steroid therapy. Granulocytic epithelial lesion (GEL) positive AIP patients differ from GEL negative AIP patients in clinical features such as equal gender ratio, younger mean age, no increase in serum IgG4, no association with extrapancreatic involvement, no relapse, and frequent association with inflammatory bowel disease. Further investigation is needed to clarify the pathogenic mechanisms including more definite serological markers for theses two entities.
Autoimmune Diseases/*diagnosis/pathology/ultrasonography ; Common Bile Duct/radiography ; Fibrosis/pathology ; Humans ; Immunoglobulin G/blood ; Pancreatitis/*diagnosis/pathology/ultrasonography ; Salivary Glands/pathology

BACKGOUND/AIMS: The involvement of bile ducts is frequently reported in autoimmune pancreatitis (AIP), which seem to have similar features to primary sclerosing cholangitis (PSC). Recent systematic comparative studies about these diseases are rare in Korea. METHODS: We retrospectively analyzed 26 patients with AIP with bile duct involvement and 30 patients with classic PSC who were diagnosed during the last decade. RESULTS: The mean age of patients was significantly higher in AIP than PSC at the time of diagnosis. There was a preponderance of men in both group, which was more prominent in AIP. The most common symptom in patients with AIP was jaundice, but PSC patients usually visited hospitals due to incidentally detected abnormal liver function tests. Most (26/31) of AIP had bile duct involvement. All of these patients showed narrowing of intrapancreatic common bile ducts and one patient exhibited hilar involvement as well. About 80% of PSC had both intra- and extrahepatic ducts involvement, and the characteristic features involve multifocal strictures. AIP patients showed improvement with steroid treatment, however, most PSC patients showed clinical deterioration. CONCLUSIONS: The clinical and cholangiographic findings of patients with AIP and PSC have many different characteristics. Therefore, further study of two diseases is required for the proper diagnosis and management.
Autoimmune Diseases/*diagnosis/pathology/radiography ; Bile Ducts/pathology ; *Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/*diagnosis/pathology/radiography ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulins/blood ; Male ; Pancreatitis/*diagnosis/*immunology/pathology ; Retrospective Studies ; Sex Factors

Immunoglobulin G4 (IgG4)-related sclerosing disease is rare and is known to involve various organs. We present a case of histologically proven IgG4-related sclerosing disease of the small bowel with imaging findings on computed tomography (CT) and small bowel series. CT showed irregular wall thickening, loss of mural stratification and aneurysmal dilatation of the distal ileum. Small bowel series showed aneurysmal dilatations, interloop adhesion with traction and abrupt angulation.
Adult ; Antibodies, Anti-Idiotypic/immunology ; Autoimmune Diseases/*diagnosis/immunology ; Humans ; Immunoglobulin G/*immunology ; Intestine, Small/*pathology/radiography ; Male ; Multidetector Computed Tomography/*methods ; Sclerosis/diagnosis/immunology

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