Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 23-year-old Japanese man was admitted to our hospital because of dry cough and lacrimal gland swelling. Laboratory findings showed hypergammaglobulinemia (4859 mg/dl), hypocomplementemia (CH50 13 U/ml), and hyperamylasemia. CT revealed a marked swelling of the bilateral lacrimal glands, diffuse patchy infiltration in the bilateral lung fields, and enlargement of the whole pancreas. Gallium citrate scintigraphy showed abnormal accumulation in the bilateral lacrimal glands, submandibular glands, and both lung fields. Biopsy specimens from the salivary gland revealed dense lymphoplasmacytic infiltration and abundant IgG4-positive plasma cells. Furthermore, open lung biopsy showed a marked peribronchial lymphoplasmacytic and eosinophilic infiltration with IgG4-positive plasma cells. These findings fulfilled the criteria of Mikulicz's disease and autoimmune pancreatitis, and support the recently proposed concept of IgG4-related systemic disease.
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PMID:[A case of bronchiolitis with IgG4-positive plasma cells infiltration associated with Mikulicz's disease and autoimmune pancreatitis]. 1926 May 38

Solid pseudopapillary tumor (SPT) of the pancreas is a rare pancreatic tumor mostly seen in young women. We here report a twelve-year-old girl presenting with recurrent attacks of pancreatitis. No history of a systemic disease, trauma, drug usage or infection was present. All other etiologic factors like familial, hypertriglyceridemia, hypercalcemia, cystic fibrosis, medications were excluded. On abdominal ultrasound a heterogeneous mass was noticed at the tail of pancreas. Computerized tomography and magnetic resonance imaging proved that the mass was cystic. The mass was surgically removed. The diagnosis was pancreatic solid cystic papillary epithelial neoplasm. Although acute pancreatitis due to SPT was exceptionally reported, this is the first description of SPT leading to recurrent pancreatitis especially in children.
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PMID:Solid pseudopapillary tumor of the pancreas as a cause of recurrent pancreatitis. 1931 80

IgG4-associated cholangitis (IAC) is a recently defined disease entity which shares a number of clinical, biochemical, and radiological features with primary sclerosing cholangitis (PSC). In contrast to PSC, IAC responds to immunosuppressive treatment, is not associated with inflammatory bowel disease, and mainly affects elderly men above the age of 60 years. Today, IAC is regarded as one variant of IgG4-related systemic disease (ISD) of which autoimmune pancreatitis (AIP) is the best studied organ manifestation. The diagnosis of IAC is based on biochemical, radiological and histologic features, among which elevated serum levels of IgG4, extra- and intrahepatic biliary strictures as visualized by cholangiography, multifocal IgG4-rich lymphoplasmacytic sclerosing infiltrations in liver and bile duct tissue, and association with AIP are of key importance. This review aims at summarizing clinical features, diagnostic criteria, therapeutic strategies and most recent insights in the pathophysiology of IAC and other organ manifestations of ISD.
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PMID:Immunoglobulin G4-associated cholangitis: one variant of immunoglobulin G4-related systemic disease. 1939 Jan 94

Since Sarles et al. [Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas-an autonomous pancreatic disease? Am J Dig Dis 1961; 6: 688-698.] reported a case of pancreatitis associated with hypergammaglobulinemia, many cases have been described, which led to the current concept of "autoimmune pancreatitis (AIP)". Lymphoplasmacytic infiltration and fibrosis on histology together with elevated IgG levels or the presence of autoantibodies on laboratory examinations supported the concept of AIP. In recent years, based on histological and immunohistochemical examination of various organs of patients with AIP, a novel clinicopathological entity, IgG4-related slerosing disease, has been proposed. AIP is a systemic disease that is characterized by dense infiltration of IgG4-positive plasma cells and T lymphocytes in various organs. Clinical manifestations are related with pancreas dysfunction but other organs may be affected such as bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung and prostate. Increased serum IgG4 levels, the presence of several autoantibodies such as anti-carbonic anhydrase II antibodies (ACA-II), immunostaining IgG4 positive in pancreas tissue and a very good response to steroid therapy are useful for the diagnosis of AIP that can mimic pancreatic cancer.
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PMID:Autoimmune pancreatitis: an underdiagnosed autoimmune disease with clinical, imaging and serological features. 1962 98

A 74-year-old male patient presented with progressive anorexia, cholestatic liver function tests, and a diffuse enlarged pancreas suggestive of a pancreatic carcinoma. There was a marked elevation of total immunoglobulin G4 (IgG4) in serum. Further investigation led to the diagnosis of IgG4-related sclerosing disease with involvement of the pancreas, retroperitoneal fibrosis, and bilateral focal nephritis. To our knowledge, this is the first report on these 3 clinical entities occurring in the same patient.A short review of the literature concerning autoimmune pancreatitis and retroperitoneal fibrosis is made, with special interest to the concept of IgG4-related pathology. This systemic disease can have several clinical manifestations: IgG4-positivity not only can be found in the pancreas, but also at the level of extrahepatic biliary ducts, gallbladder, salivary glands, retroperitoneal tissue, kidneys, ureters, and lymph nodes. Although further investigation is required to determine its exact pathophysiologic role, IgG4 seems to be an important key player.
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PMID:A case of IgG4-related sclerosing disease with retroperitoneal fibrosis, autoimmune pancreatitis and bilateral focal nephritis. 1989 55

Both multifocal fibrosclerosis and hypertrophic pachymeningitis are rare disorders of unknown etiology, characterised by chronic inflammation leading to dense fibrosis. There have been several reports of multifocal fibrosclerosis with hypertrophic pachymeningitis. Autoimmune pancreatitis is frequently associated with various extrapancreatic lesions, their pathological similarities such as dense inflammatory fibrosis with lymphoplasmacytic infiltration strongly suggests a close relationship between autoimmune pancreatitis and multifocal fibrosclerosis. Recently, autoimmune pancreatitis including these systemic fibrosing disorders may be classified as IgG4-related systemic disease. However, the relationship between HP and IgG4-related systemic disease is still uncertain. We performed immunohistochemical examinations in autopsy specimens from a patients with HP. Histological findings can be summarized as follows: sever interstitial fibrosis and diffuse inflammatory cells infiltration, presenting nonspecific inflammatory changes. Immunohistochemically, diffuse infiltrates in the dura consisted predominantly of UCHL-1 positive T or L-26 positive B lymphocytes. Many IgG4 positive plasma cells were also infiltrated. To our knowledge, this may be the first report which showed IgG4 positive plasma cells infiltration in the dura in a patient with HP. It is postulated that HP may be a dural lesion of IgG4-related systemic disease.
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PMID:[Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease?]. 1992 92

Autoimmune pancreatitis (AIP) is a peculiar type of pancreatitis of presumed autoimmune etiology. AIP is diagnosed currently based on characteristic radiological findings (irregular narrowing of the main pancreatic duct and enlargement of the pancreas) in combination with serological findings (elevation of serum IgG, and IgG4, and presence of autoantibodies), clinical findings (elderly male preponderance, occasional extrapancreatic lesions, and favorable response to steroid therapy), and histopathological findings (dense infiltration of IgG4-positive plasma cells and T lymphocytes with fibrosis and obliterative phlebitis in the pancreas). Based on histological and immunohistochemical examination of various organs of AIP patients, a novel clinicopathological entity of "IgG4-related sclerosing disease" has been proposed. This is a systemic disease that is characterized by extensive IgG4-positive plasma cell and T lymphocyte infiltration of various organs. Clinical manifestations are apparent in organs such as the pancreas, bile duct, gallbladder, salivary or lacrimal gland, and retroperitoneum, where tissue fibrosis with obliterative phlebitis is pathologically induced. AIP is not simply a form of pancreatitis but rather a pancreatic lesion reflecting an IgG4-related sclerosing disease. This disease includes autoimmune pancreatitis, IgG4-related sclerosing cholangitis, IgG4-related cholecystitis, IgG4-related sialadenitis, IgG4-related retroperitoneal fibrosis. In some cases, only 1 or 2 organs are clinically involved, while in others 3 or 4 organs are affected. Serum IgG4 levels and immunostaining with anti-IgG4 antibody are useful in establishing the diagnosis. Since malignant tumors are frequently suspected on initial presentation, IgG4-related sclerosing disease should be considered in the differential diagnosis to avoid unnecessary surgery.
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PMID:[IgG4-related sclerosing disease]. 2003 Jan 80

A new concept of IgG subclass such as IgG4-related systemic disease including autoimmune pancreatitis, characterized by a high-serum IgG4 level, has been proposed. Our aim was to investigate serum IgG subclass levels in rheumatoid arthritis (RA). IgG subclass levels of sera from 72 patients with rheumatoid arthritis were determined by immunonephelometric assay. The patients were divided into two groups according to clinical activity of the disease: active disease and remission. The sera from 45 normal controls were also measured. All statistical analyses were carried out with SPSS software version 13.0. We found a significant increase of all the four IgG subclass concentrations in sera of patients with RA compared to those of the controls (P < 0.001). When the patients were divided according to clinical activity, the IgG subclass concentrations were similar between the two groups (P > 0.05). Our data suggest that besides IgG1, IgG2 and IgG3, IgG4 may involve in the pathogenesis of the disease.
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PMID:Elevation of serum IgG subclass concentration in patients with rheumatoid arthritis. 2005 78

A 78-year-old-man was admitted to our hospital because of renal insufficiency 20 months after the onset of autoimmune pancreatitis. He had cerebral infarction and prostatic hypertrophy as complications. He had been previously diagnosed with autoimmune pancreatitis (AIP). The initial therapy was started with oral prednisolone at the dose of 0.8 mg/kg (40 mg/day). Prednisolone had been tapered off gradually through a one-year period. Four months later from terminating prednisolone, a follow-up CT showed multiple low-density areas in both kidneys without swelling of the pancreas. Furthermore, 4 months later, laboratory findings showed progressive renal insufficiency. On admission, BP was 167/77 mmHg, and the bilateral submaxillary glands were swollen. He did not have pretibial edema. Laboratory findings were as follows. BUN 55.9 mg/dL, Cre 6.17 mg/dL, Amy 65 mg/dL, TP/Alb 9.5/4 g/dL, gamma-gl 43.7%, IgG/IgA/IgM 3,395/112/74 mg/dL, IgG4 1,460 mg/dL, urinary protein 1.38 g/day, and 24 hr-Ccr 11.8 mL/min/1.73 m2. Percutaneous renal needle biopsy was conducted. Light microscopic findings demonstrated tubulointerstitial nephritis (TIN) and membranous change. Immunofluorescent microscopic findings indicated diffuse deposition of IgG2 and IgG4 in the renal interstitium. On the basis of these findings, the condition was diagnosed as IgG4-related tubulointerstitial nephritis. As renal insufficiency was progressing, hemodialysis was started soon after admission and oral prednisolone was also started at the dose of 0.4 mg/kg (20 mg/day). However, improvement of renal function has not been obtained and hemodialysis and prednisolone tapering are still being conducted. This case showed severe tubulointerstitial nephritis requiring hemodialysis after a cure for autoimmune pancreatitis. IgG4-related renal disease rarely needs hemodialysis. This case indicates that the prognosis of IgG4-related systemic disease is not necessarily good and further accumulation of cases is required.
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PMID:[Case of IgG4-related tubulointerstitial nephritis showing the progression of renal dysfunction after a cure for autoimmune pancreatitis]. 2016 45

Autoimmune pancreatitis (AIP) is a type of chronic pancreatitis characterized by swelling of the pancreas, narrowing of the main pancreatic duct, elevation of serum immunoglobulin G or G4 level or presence of several autoantibodies, or lymphoplasmacytic infiltration and fibrosis in the pancreas. However, the pathogenesis of AIP remains unclear, and the natural history and long-term prognosis of AIP are little known. Oral corticosteroid therapy for AIP is recommended. The absolute indications for steroid therapy for AIP are bile duct stenosis and accompanying systemic disease such as retroperitoneal fibrosis and diabetes mellitus. The dosage for remission induction is 30 to 40 mg/d for 1 to 2 months. The remission maintenance is needed to prevent relapse, and 5 to 10 mg/d for at least 6 months is recommended in patients who do not have complete remission. When relapse occurs, the dose used at remission induction can be readministered. Herein, we discuss remission and relapse of AIP, focusing on corticosteroid treatment to help clinicians care for patients with AIP and to help make an ideal treatment protocol of AIP through a review of published data. We tried to define remission and relapse of AIP to help investigate the natural course of AIP.
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PMID:Remission and relapse of autoimmune pancreatitis: focusing on corticosteroid treatment. 2018 97


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