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)

Systemic lupus erythematosus is a complex immunological and rheumatological disease that has numerous complications. Central to the pathogenesis of systemic lupus erythematosus is immune complex formation and deposition in blood vessels and end organs. This is a case report of an autopsy of a patient with systemic lupus erythematosus, end stage renal disease, peripheral vascular occlusive disease, pancreatitis, and aortitis. The aortitis was found to be immune complex mediated with deposition of IgG, C3, as well as fibrinogen in the wall of the aorta as shown by immunofluorescence. The hypercoagulable state of the patient is discussed with particular emphasis on the role of anticardiolipin antibodies, antiphospholipid antibodies, and anti-beta-2-glycoprotein I in the pathogenesis. This case is unique in that the immune complex mediated aortitis has not been described in the literature over the past 25 years. We recommend that the diagnosis of immune complex mediated aortitis be considered in the differential diagnosis of aortitis, particularly in the background of a patient with systemic lupus erythematosus.
 ...
PMID:Lupus aortitis: a case report and review of the literature. 874 62

Several paraneoplastic inflammatory conditions, particularly autoimmune diseases, have been described in association with myelodysplastic syndromes (MDS). However, to date, recurrent acute pancreatitis has never been described in association with MDS. A 44-year-old man presented with prolonged fever and fatigue. Aortitis and pericarditis were diagnosed simultaneously with MDS, refractory anemia with excess blast type 2. His erythrocyte sedimentation rate and c-reactive protein were markedly elevated. The vasculitic syndrome responded rapidly to corticosteroids, but soon after tapering of corticosteroids, acute pancreatitis developed. Pain and pancreatic enzymes, however, improved rapidly with escalation of corticosteroid dosage. Multiple attempts at discontinuing the drug resulted in symptomatic flare-ups. Finally, his MDS transformed into acute myeloid leukemia (AML); severe acute pancreatitis closely accompanied. Induction chemotherapy and high-dose corticosteroids, however, controlled both conditions. A subsequent pancreatitis attack with pseudocyst formation occurred, but again was controlled with corticosteroids, although this was followed closely by another relapse of AML. All etiologies for recurrent acute pancreatitis were ruled out. The dramatic response of his pancreatitis attacks to immunosuppression suggested its autoimmune origin, while the close relationship in both the timing and severity of acute pancreatitis and MDS/AML suggested that the autoimmune pancreatitis was a paraneoplastic phenomenon related to MDS.
 ...
PMID:Recurrent steroid-responsive pancreatitis associated with myelodysplastic syndrome and transformations. 1562 95

Hyper-IgG4 syndrome, or IgG4-related systemic disease (IgG4-RSD), has been recently characterized by the association of a focal or diffuse enlargement in one or more organs, elevated levels of serum IgG4 and histopathological findings including "storiform" fibrosis and prominent infiltration of lymphocytes and IgG4-positive plasma cells. Pancreas was the first organ involved with sclerosing pancreatitis (or autoimmune pancreatitis). Since this first description, many extrapancreatic lesions have been described, even in the absence of pancreatitis and include sialadenitis, lacrimal gland inflammation, lymphadenopathy, aortitis, sclerosing cholangitis, tubulointerstitial nephritis, retroperitoneal fibrosis or inflammatory pseudotumors. Multiorgan lesions can occur synchronously or metachronously in a same patient, usually after 50 years of age. They all share common histopathological findings. The disease often responds well to corticosteroid therapy. In this literature review on IgG4-RSD, we present historical, epidemiological and clinical characteristics, and we review the biological and histological diagnostic criteria. To date there is no international validated diagnostic criteria. Pathophysiological hypothesis and therapeutic approaches are also discussed.
 ...
PMID:[IgG4-related systemic disease: emergence of a new systemic disease? Literature review]. 2195 22

IgG4 related disease is a chronic immunopathological disorder, proposed as a new diagnostic entity, and characterized as follows: 1. background is in multiorgan lymphoplasmocellular infiltration associated with presence of large number of polyclonal IgG4+ plasma cells, and fibrosclerosis; 2. structural damage of the affected organs is frequently associated with pseudotumorous enlargement and/or other morphological changes; for example aortitis with aneurysmatic dilatation, sclerosing pancreatitis, retroperitoneal fibrosis ect.; 3. serum concentration of IgG4 is usually significantly elevated; 4. responsivity to glucocorticoids is a hallmark of the disease: in rare cases of glucocorticoid-resistance a response to rituximab was observed. At presence the disease related to IgG4 is considered as a rare disorder. Presented knowledge may be useful for better elucidation of its importance in clinical specialities.
 ...
PMID:[Clinical importance of the IgG4 related disease]. 2202 78

IgG4-related disease is an emerging pathological condition characterized by one or several fibrosing and inflammatory organ involvements. Histological findings are typical and associate storiform fibrosis with polyclonal lymphocytic and plasma-cell infiltrate, with predominant IgG4-expressing plasma cells. Sclerosing lymphoplasmocytic pancreatitis, or type 1 auto-immune pancreatitis, and other organ involvements have been reported: sclerosing cholangitis, sialadenitis, dacryoadenitis, retroperitoneal fibrosis, aortitis, interstitial nephritis, polyadenopathy and inflammatory pseudo-tumors. Serum IgG4 level is elevated in most of patients, but the histological documentation remain necessary for the diagnosis. Dramatic response to steroids is usual but relapses are frequent.
 ...
PMID:[IgG4-related disease]. 2378 81

Immunoglobulin4 (IgG4)-related disease is a systemic inflammatory disease characterized by elevation of serum IgG4. It involves various organs such as the pancreas (autoimmune pancreatitis), lacrimal gland (Mikulicz's disease), retroperitoneum (retroperitoneal fibrosis), aorta (aortic aneurysm and aortitis), heart (constrictive pericarditis), and pseudotumors around the coronary arteries. These disorders often coexist in accordance with progression of the disease. Because IgG4-related cardiovascular disorder affects the patient's prognosis, early detection and treatment is important. Coronary CT imaging and echocardiography accidentally detect IgG4-related disorders and (18)FDG-PET imaging can identify active inflammation in the lesions. Measurement of serum IgG4 levels and tissue biopsy are necessary for diagnosis. Minor salivary gland biopsy is recommended even though (18)FDG uptake is not detected when it is difficult to obtain a biopsy specimen from IgG4-related cardiovascular lesions. The first-line treatment is high-dose corticosteroid therapy, however, relapse is often reported. Corticosteroids suppress the development of active inflammatory diseases such as aortitis, pericarditis, and pseudotumors, but already-developed lesions do not respond. A large developed aneurysm can rupture even during or after corticosteroid therapy, therefore, additional surgical treatment may be needed. Treatment of IgG4-related cardiovascular disorders might require higher doses of corticosteroids than IgG4-related extracardiovascular disorders. The adequate dose of corticosteroid, type and dose of immunosuppressant, and surgical intervention should be carefully considered on a case-by-case basis.
...
PMID:IgG4-related cardiovascular disorders. 2489 99

Immunoglobulin (IgG) 4-related disease is a systemic inflammatory disease, and it affects vascular system as aortitis, periaortitis, or aneurysm. However, due to a lack of serum biomarker on aortic damage and the multiorgan involvement, it is difficult to assess aortic inflammatory activity of IgG4-related disease. We described a case of IgG4-related pancreatitis and aortitis, which was visualized with magnetic resonance merged image of diffusion weighted and T1 weighted images. The aortic signal intensity or apparent diffusion coefficient value reduced or increased after oral prednisone administration, respectively. Magnetic resonance diffusion weighted image and apparent diffusion coefficient may be a useful imaging tool for assessment of vascular inflammation in IgG4-related aortitis.
 ...
PMID:Therapeutic response of immunoglobulin 4-related aortitis and pancreatitis demonstrated by diffusion-weighted MRI. 3136 Feb 74

Objectives: Immunoglobulin G4-related disease (IgG4-RD) is a systemic, multiorgan disease of unknown etiology. We aimed to classify IgG4-RD by a combination pattern of affected organs and identify the clinical features, including the comorbidities of each subgroup.Methods: Patients diagnosed with IgG4-RD between April 1996 and June 2018 were enrolled from three institutes. Hierarchical cluster analysis was performed using six frequently affected organs (lacrimal gland and/or orbit, salivary gland, lung, pancreas, kidney, and retroperitone and/or aorta). Clinical features, such as comorbidities and outcomes, were compared between clusters.Results: In total, 108 patients enrolled in this cohort could be stratified into five distinct subgroups: group 1, lung dominant group; group 2, retroperitoneal fibrosis and/or aortitis dominant group; group 3, salivary glands limited group; group 4, Mikulicz's disease dominant group; and group 5, autoimmune pancreatitis with systemic involvement group. There were significant between-group differences in sex (male dominant in group 1, 2, and 5), history of asthma and allergies on the respiratory tract (most frequent in group 5), and malignancy (most frequent in group 5).Conclusion: IgG4-RD can be classified into subgroups according to the pattern of affected organs. Group 5 may have frequent complications with allergies and malignancies.
 ...
PMID:Phenotyping of IgG4-related diseases based on affected organ pattern: A multicenter cohort study using cluster analysis. 3190 17

We present an unusual case of syphilitic pancreatitis and ascending aortitis in a 41-year-old HIV-negative male patient presenting to a tertiary institution with obstructive jaundice. After a battery of investigations that included computed tomography (CT) and 18F-labelled fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, syphilis serology and histology, a diagnosis of tertiary syphilis was made. The patient responded favourably to antibiotics, with resolution of all lesions on FDG PET/CT 13 weeks after initiation of therapy. Even though tertiary syphilis is a rare entity, it should be earmarked as a mimicker of other pathological conditions, including, in this case, primary pancreatic malignancy.
 ...
PMID:Syphilitic pancreatitis: A rare mimicker of our time. 3288 Mar 40

Thoracic and abdominal pathology are common in the emergency setting. Although computed tomography is preferred in many clinical situations, magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) have emerged as powerful techniques that often play a complementary role to computed tomography or may have a primary role in selected patient populations in which radiation is of specific concern or intravenous iodinated contrast is contraindicated. This review will highlight the role of MRI and MRA in the emergent imaging of thoracoabdominal pathology, specifically covering acute aortic pathology (acute aortic syndrome, aortic aneurysm, and aortitis), pulmonary embolism, gastrointestinal conditions such as appendicitis and Crohn disease, pancreatic and hepatobiliary disease (pancreatitis, choledocholithiasis, cholecystitis, and liver abscess), and genitourinary pathology (urolithiasis and pyelonephritis). In each section, we will highlight the specific role for MRI, discuss basic imaging protocols, and illustrate the MRI features of commonly encountered thoracoabdominal pathology.
 ...
PMID:Role of MRI in the Evaluation of Thoracoabdominal Emergencies. 3326 75


1