Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0149521 (chronic pancreatitis)
 7,199 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Mice with the homozygous mutation alymphoplasia (aly) lack lymph nodes and Peyer's patches and show defects in both humoral and cellular immunity. In these mice, spontaneous infiltration of mononuclear cells was observed in multiple exocrine organs, including the pancreas, salivary glands, and lacrimal glands from the age of 15 wk, progressing to a marked tissue destruction at the age of 25 wk. Using this strain, we examined the phenotypes and TCR Vbeta gene expression of infiltrating T cells to identify the pathologic role of T cell immunity in idiopathic pancreatitis. Most of the infiltrating cells were CD4+ and Thy-1+ cells. Analysis of the TCR gene expression on T cells infiltrating the pancreas and salivary glands showed a high expression of Vbeta1 and Vbeta5 in both organs at the age of 15 wk. In contrast, a diverse expression of TCR Vbeta genes was noted at 25 wk. Sequence analysis of complementarity-determining region 3 (CDR3) of the most prominent TCR Vbeta gene family expressed in these cells, Vbeta1, showed oligoclonal expansion of infiltrating T cells in both organs. Frequent use of glutamine and proline at position 97 was observed in paired tissues. Our data suggest that oligoclonal expansion of organ specific T cells might be one of the etiologic mechanisms of chronic pancreatitis and that common autoantigens could trigger autoimmunity in multiple organs.
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PMID:Shared TCR Vbeta gene expression by the pancreas and salivary gland in immunodeficient alymphoplasic mice. 920 Apr 82

Autoimmune pancreatitis (AIP) is responsive to steroid therapy, but some AIP patients improve spontaneously, or only improve after biliary drainage. Pancreatic enlargement and irregular narrowing of the main pancreatic duct usually improves in almost all patients, but marked atrophy of the pancreas develops in some patients. Biliary stenosis improves to various degrees, and a biliary drainage tube can be withdrawn. Other extrapancreatic lesions, including swelling of the salivary or lacrimal glands, lymphadenopathy, and retroperitoneal fibrosis also improve with steroid therapy. Pancreatic endocrine and exocrine function is frequently impaired in AIP patients, and steroid therapy is occasionally effective for these dysfunction. Deterioration of pancreatic exocrine function is rarely detected after steroid therapy. In the literature, the recurrence rate of AIP was reported to be about 17% (range 6% to 26%). AIP patients who relapse during maintenance steroid therapy or after stopping steroid medication should be re-treated with a high-dose steroid. Although AIP is rarely associated with pancreatic stones, stones are formed in some relapsing AIP patients. The long-term prognosis for AIP is unknown. As the pancreatic exocrine and endocrine functions as well as the morphological findings are reversible after steroid therapy, the prognosis for AIP seems better than that of chronic pancreatitis, which is usually followed by exocrine and endocrine pancreatic insufficiency with disease progression. Although carcinogenesis of AIP is unknown, some AIP patients developed a malignancy during follow-up. Since AIP occurs predominantly in the elderly, clinicians should pay attention to any complicating diseases in follow-up of AIP patients. Further studies are necessary to clarify the pathogenesis as well as the long-term prognosis of AIP.
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PMID:Prognosis of autoimmune pancreatitis. 1752 Feb 25

Autoimmune pancreatitis is the pancreatic manifestation of a systemic disorder that affects various organs, including the bile duct, retroperitoneum, kidney, and parotid and lacrimal glands. It represents a recently described subset of chronic pancreatitis that is immune mediated and has unique histologic, morphologic, and clinical characteristics. A hallmark of the disease is its rapid response to corticosteroid treatment. Although still a rare disease, autoimmune pancreatitis is increasingly becoming recognized clinically, leading to evolution in the understanding of its prognosis, clinical characteristics, and treatment.
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PMID:Autoimmune pancreatitis. 1849 30

Before the concept of autoimmune pancreatitis (AIP) was established, this form of pancreatitis had been recognized as lymphoplasmacytic sclerosing pancreatitis or non-alcoholic duct destructive chronic pancreatitis based on unique histological features. With the discovery in 2001 that serum IgG4 concentrations are specifically elevated in AIP patients, this emerging entity has been more widely accepted. Classical cases of AIP are now called type 1 as another distinct subtype (type 2 AIP) has been identified. Type 1 AIP, which accounts for 2% of chronic pancreatitis cases, predominantly affects adult males. Patients usually present with obstructive jaundice due to enlargement of the pancreatic head or thickening of the lower bile duct wall. Pancreatic cancer is the leading differential diagnosis for which serological, imaging, and histological examinations need to be considered. Serologically, an elevated level of IgG4 is the most sensitive and specific finding. Imaging features include irregular narrowing of the pancreatic duct, diffuse or focal enlargement of the pancreas, a peri-pancreatic capsule-like rim, and enhancement at the late phase of contrast-enhanced images. Biopsy or surgical specimens show diffuse lymphoplasmacytic infiltration containing many IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. A dramatic response to steroid therapy is another characteristic, and serological or radiological effects are normally identified within the first 2 or 3 weeks. Type 1 AIP is estimated as a pancreatic manifestation of systemic IgG4-related disease based on the fact that synchronous or metachronous lesions can develop in multiple organs (e.g. bile duct, salivary/lacrimal glands, retroperitoneum, artery, lung, and kidney) and those lesions are histologically identical irrespective of the organ of origin. Several potential autoantigens have been identified so far. A Th2-dominant immune reaction and the activation of regulatory T-cells are assumed to be involved in the underlying immune reaction. IgG4 antibodies have two unique biological functions, Fab-arm exchange and a rheumatoid factor-like activity, both of which may play immune-defensive roles. However, the exact role of IgG4 in this disease still remains to be clarified. It seems important to recognize this unique entity given that the disease is treatable with steroids.
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PMID:Type 1 autoimmune pancreatitis. 2215 22

Autoimmune pancreatitis (AIP) is a rare disease with clinical presentations that greatly mimic pancreatic cancer (PC). It is critical for clinicians to distinguish AIP from PC because their treatments and prognoses are entirely different. Typical images show characteristic features such as diffuse pancreatic swelling and strictures of the main pancreatic duct (MPD). However, AIP may present as a localized pancreatic mass, in which case it is very difficult to differentiate from PC. Here, we report a case of a 40-year-old man with computed tomography (CT) imaging studies confirming an area of low-density neoplasm in the uncinate process of the pancreas with dilation in the common biliary duct (CBD) and MPD. Increased uptake in the uncinate mass was observed by positron emission tomography (PET)/CT scan, which strongly suggested PC. Further laboratory analyses showed a marked elevation of serum IgG4. Because there was not enough evidence to rule out a diagnosis of malignancy, a histopathological biopsy became the criterion standard. An endoscopic ultrasound (EUS)-guided needle biopsy failed. As an alternative, a pancreaticoduodenectomy was conducted for the biopsy, and pathological analysis confirmed IgG4-related sclerotic chronic pancreatitis with moderate lymphoplasmacellular infiltration.We suggest that an accurate preoperative diagnosis for localized AIP with MPD and CBD obstructions mimicking PC is of great importance. Radiological imaging findings, particularly observations of diffused enlargement of the pancreas and delayed enhancement during the venous and portal phases, are essential for diagnosing AIP. Careful consideration should be given if serum IgG4 was taken as a special indicator for a differential diagnosis between AIP and PC. A history of IgG4-related diseases involving the biliary, lacrimal, salivary, retroperitoneal, renal, or pulmonary systems should also be highlighted. Thus, the pathology of extrapancreatic organs can be utilized as diagnostic evidence when the pathology for a pancreatic mass is not available, as in the case presented here. Furthermore, cautious use of hormone therapy is indicated for patients who cannot be ruled out as having PC. The results of future studies on localized AIP are eagerly awaited.
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PMID:Localized Autoimmune Pancreatitis: Report of a Case Clinically Mimicking Pancreatic Cancer and a Literature Review. 2649 72