Autoimmune Diseases ; diagnosis ; immunology ; pathology ; therapy ; Child ; China ; Gastroenterology ; Hepatitis B ; diagnosis ; pathology ; therapy ; Humans ; Infant

IgG4-related disease (IgG4-RD) is an immune-mediated condition associated with chronic fibroinflammatory lesions that can affect nearly any organ. IgG4-related hepatobiliary and pancreatic diseases are IgG4-RD involving the hepatobiliary and pancreatic system, which is characterized with elevated serum IgG4 concentrations, large numbers of IgG4 positive lymphoplasma cells infiltration in affected organs, storiform fibrosis, and imaging changes of organ morphology. Due to the lack of reliable biomarkers, histopathology is still an important basis for diagnosis. The pathogenesis of IgG4-related hepatobiliary and pancreatic diseases has not been clarified. This review focuses on the recent advances in intestinal microecology-immunology, host genetics-immunity and recurrence monitoring of IgG4-related hepatobiliary and pancreatic diseases.
Autoimmune Diseases/diagnosis* ; Gastrointestinal Microbiome ; Humans ; Immunoglobulin G ; Immunoglobulin G4-Related Disease/pathology* ; Pancreatic Diseases

No abstract available.
Autoimmune Diseases/*diagnosis/therapy ; Humans ; Immunoglobulin G/blood ; Pancreatitis, Chronic/*diagnosis/pathology/therapy

No abstract available.
Autoimmune Diseases/*diagnosis/therapy ; Humans ; Immunoglobulin G/blood ; Pancreatitis, Chronic/*diagnosis/pathology/therapy

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

Myasthenia gravis is a chronic disease characterized by skeletal muscle weakness. The etiology of myasthenia gravis is not clarified but recently it has been suggested that it is an autoimmune disease. Myasthenia gravis is not difficult to diagnose but the therapy remains as an assignment. The author observed in 10 cases of myasthenia gravis attending the eye department of B.N.U. hospital for 5 years from 1967 to 1971, and review of the literature of recent yeary related to myasthenia gravis, particulary its pathogenesis, clinical course, pathology, physiology, diagnosis, treatment, and prognosis were discussed.
Autoimmune Diseases ; Chronic Disease ; Diagnosis ; Muscle, Skeletal ; Myasthenia Gravis* ; Pathology ; Physiology ; Prognosis

This study was aimed to investigate the clinical characteristics and treatment of patients with autoimmune disease combined with non-Hodgkin lymphoma (NHL). The clinical characteristics and pathologic patterns of 6 patients with NHL who concurrently suffered from autoimmune diseases were analysed retrospectively from aspects of clinical course, pathologic features, and therapy. Treatment outcomes for autoimmune diseases and NHL were observed. The results showed that 6 patients included 4 females and 2 males, range in age from 28 to 65 years with a median age of 56 years. The autoimmune diseases are Sjogren's syndrome (SS, 2 cases), rheumatoid arthritis (RA, 2 cases), ulcerative colitis (UC, 1 case) and Crohn's disease (CD, 1 case). The NHL diseases located not only in the lymph node (n = 3) but also in extranodal sites (n = 3). Histologically, 3 cases were diffuse large B cell lymphoma (DLBCL), 2 cases were extranodal nasal NK/T lymphoma (ENKL) and 1 case was peripheral T cell lymphoma, not otherwise specified. Based on CD10, Bcl-6 and MUM1 expression patterns, all 3 DLBCL were classified as non-GC subtype. EBER positive tumor cells were detected in 2 case of ENKL. 5 patients achieved a complete remission (83%) and 1 patient was primary drug-resistant after CHOP chemotherapy or involved radiotherapy. Median survival from the time of lymphoma diagnosis was 3 years. 1 patient showed clinical improvement of the SS symptoms, 2 patients (CD and UC) showed stable state of disease and 2 patients with RA and 1 patient with SS needed continuing treatment for their autoimmune diseases after chemotherapy for NHL. It is concluded that the development of NHL is one of the most serious complications in patients with autoimmune diseases. There is an increased frequency of non-GC subtype DLBCL. CHOP combined with or without radiotherapy proves to be effective for autoimmune disease patients with aggressive NHL but ineffective for concurrent autoimmune diseases.
Adult ; Aged ; Autoimmune Diseases ; diagnosis ; pathology ; therapy ; Female ; Humans ; Lymphoma, Non-Hodgkin ; diagnosis ; pathology ; therapy ; Male ; Middle Aged ; Retrospective Studies

BACKGROUND/AIMS: Diffuse or segmental irregular narrowing of the main pancreatic duct (MPD), as observed by endoscopic retrograde cholangiopancreatography (ERCP), is a characteristic feature of autoimmune pancreatitis (AIP). METHODS: ERCP findings were retrospectively examined in 40 patients with AIP in whom irregular narrowing of the MPD was detected near the orifice. The MPD opening sign was defined as the MPD within 1.5 cm from the orifice being maintained. The distal common bile duct (CBD) sign was defined as the distal CBD within 1.5 cm from the orifice being maintained. Endoscopic findings of a swollen major papilla and histological findings of specimens obtained from the major papilla were examined in 26 and 21 patients, respectively. RESULTS: The MPD opening sign was detected in 26 of the 40 patients (65%). The distal CBD sign was detected in 25 of the 32 patients (78%), which showed stenosis of the lower bile duct. The patients who showed the MPD opening sign frequently showed the distal CBD sign (p=0.018). Lymphoplasmacytic infiltration, but not dense fibrosis, was histologically detected in biopsy specimens obtained from the major papilla. CONCLUSIONS: On ERCP, the MPD and CBD adjacent to the major papilla are frequently maintained in patients with AIP involving the pancreatic head. These signs are useful for diagnosing AIP on ERCP.
Autoimmune Diseases/*diagnosis/pathology ; *Cholangiopancreatography, Endoscopic Retrograde ; Common Bile Duct/pathology ; Female ; Humans ; Male ; Middle Aged ; Pancreas/pathology ; Pancreatic Ducts/pathology ; Pancreatitis/*diagnosis/pathology ; Retrospective Studies

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