Autoimmune Pancreatitis

2018 ◽  
Author(s):  
Allison L Yang ◽  
Julia McNabb-Baltar
Keyword(s):  
Chronic Pancreatitis ◽  
Obstructive Jaundice ◽  
Autoimmune Pancreatitis ◽  
Pancreatic Insufficiency ◽  
Imaging Findings ◽  
Laboratory Findings ◽  
Related Disease

Autoimmune pancreatitis (AIP) is a subcategory of chronic pancreatitis that is highly responsive to steroids. The term was first proposed in 1995 by Yoshida and colleagues, and since its discovery, the diagnosis of AIP has dramatically increased. AIP is a chronic fibroinflammatory disease characterized by lymphoplasmacytic infiltrates and fibrosis on histology. There are two distinct subtypes: type 1 AIP is the pancreatic manifestation of a systemic serum immunoglobulin G subtype 4–related disease (IgG4-RD) and type 2 AIP is described clinically as idiopathic duct-centric pancreatitis and has no association with IgG4. Clinically, AIP presents most commonly as obstructive jaundice in type 1 AIP and can present as acute pancreatitis in type 2 AIP. The diagnostic criteria include histology, imaging findings, and responsiveness to steroids as well as laboratory findings and other organ involvement. The mainstay of treatment is steroid therapy, with immunomodulators such as rituximab used for maintenance or relapsing disease. Long-term complications of AIP include pancreatic insufficiency and are often associated with relapsing disease. This review contains 45 references, 1 figure, and 2 tables. Key Words: autoimmune pancreatitis, chronic pancreatitis, EUS-guided biopsy, IgG4, immunomodulatory, obstructive jaundice, pancreas mass, steroid

Histological differences of intrapancreatic neural system among ordinary chronic pancreatitis and autoimmune pancreatitis type 1 and type 2

Pancreatology ◽  
2017 ◽  
Vol 17 (4) ◽  
pp. S40
Author(s):  
Tsukasa Ikeura ◽  
Kota Kato ◽  
Kazushige Uchida ◽  
Makoto Takaoka ◽  
Akiyoshi Nishio ◽  
...  
Keyword(s):  
Chronic Pancreatitis ◽  
Autoimmune Pancreatitis ◽  
Neural System

scholarly journals Chronic pancreatitis and diabetes mellitus: a review of the literature

2019 ◽  
Vol 47 (6) ◽  
pp. 525-534
Author(s):  
E. Yu. Lomakina ◽  
O. V. Taratina ◽  
E. A. Belousova
Keyword(s):  
Diabetes Mellitus ◽  
Chronic Pancreatitis ◽  
Glucose Tolerance ◽  
Impaired Glucose Tolerance ◽  
Pancreatic Insufficiency ◽  
Diabetic Patients ◽  
Pancreatic Diseases ◽  
Pathophysiological Mechanisms

Background: For a long time there has been a discussion about how chronic pancreatitis (CP) and diabetes mellitus (DM) are related to each other. If a patient has both conditions, should they be viewed as two separate disorders, or one of them is a plausible consequence of the other? If the latter is true, what are pathophysiological mechanisms of DM in CP? Current consensus documents by specialists in pancreatic diseases pay little attention to this issue, and their main statements have low level of evidence. The Russian consensus on the diagnosis and treatment of CP (2016) contains no statements on DM. In the Russian guidelines and consensus documents to be developed, it is necessary to include provisions on the pancreatogenic DM as an independent “other type DM’, with consideration of its pathophysiological mechanisms and clinical particulars.Aim: To characterize the state-of-the-art in pancreatogenic DM, to demonstrate its differences from DM types 1 and 2 from pathogenetic and clinical perspectives.Methods: The review is based on the results of meta-analyses, systematic reviews and main provisions of the existing clinical guidelines and consensus documents available from PubMed and E-library.Results: According to various sources, Type 3c DM, or latent impaired glucose tolerance in CP, can eventually develop in 25 to 80% patients with CP. Impaired glucose tolerance is found in 40 to 60% of patients with acute pancreatitis, with persistent hyperglycemia after acute episode seen in 15 to 18% of the patients. Exocrine pancreatic insufficiency is commonly seen in Type 1 and Type 2 diabetic patients, although the data on its prevalence are highly contradictory indicating a lack of knowledge in the field. Type 3c DM is characterized by its manifestation at later stages of CP, concomitant excretory deficiency of the pancreas, brittle course with proneness to hypoglycemia and no ketoacidosis. The highest risk group includes patients with longstanding CP, previous partial pancreatic resection and patients with early calcifying pancreatitis, mainly of the alcoholic origin. Optimal and rational medical treatment of pancreatogenic DM still remains disputable, while the evidence base of the efficacy and safety of various anti-diabetic agents in this disease is lacking, and no consensus on the issue has been yet reached. General treatment guidelines given in a number of international consensus documents are limited to cautious insulin administration.Conclusion: Pancreatogenic DM differs from Type 1 and Type 2 DM in a number of aspects, namely, mechanisms of hyperglycemia, hormonal profiles, clinical particulars and treatment approaches. Endocrine pancreatic insufficiency in CP is caused by secondary inflammatory injury of the pancreatic islets. The key to specifics of Type 3c DM lies in anatomical and physiological interplay of the exocrine and endocrine compartments of the pancreas. At presents, most provisions on pancreatogenic DM are empirical and seem to be rather declarative, because intrinsic mechanisms of this type of diabetes and moreover its pathogenetically based treatment have been poorly studied. Nevertheless, all patients with CP or other pancreatic diseases should be assessed for pancreatogenic DM.

scholarly journals Steroid Therapy and Steroid Response in Autoimmune Pancreatitis

2019 ◽  
Vol 21 (1) ◽  
pp. 257 ◽  
Author(s):  
Hiroyuki Matsubayashi ◽  
Hirotoshi Ishiwatari ◽  
Kenichiro Imai ◽  
Yoshihiro Kishida ◽  
Sayo Ito ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Critical Role ◽  
Steroid Treatment ◽  
Clinical Images ◽  
Corticosteroid Administration ◽  
Steroid Response ◽  
Sclerosing Pancreatitis

Autoimmune pancreatitis (AIP), a unique subtype of pancreatitis, is often accompanied by systemic inflammatory disorders. AIP is classified into two distinct subtypes on the basis of the histological subtype: immunoglobulin G4 (IgG4)-related lymphoplasmacytic sclerosing pancreatitis (type 1) and idiopathic duct-centric pancreatitis (type 2). Type 1 AIP is often accompanied by systemic lesions, biliary strictures, hepatic inflammatory pseudotumors, interstitial pneumonia and nephritis, dacryoadenitis, and sialadenitis. Type 2 AIP is associated with inflammatory bowel diseases in approximately 30% of cases. Standard therapy for AIP is oral corticosteroid administration. Steroid treatment is generally indicated for symptomatic cases and is exceptionally applied for cases with diagnostic difficulty (diagnostic steroid trial) after a negative workup for malignancy. More than 90% of patients respond to steroid treatment within 1 month, and most within 2 weeks. The steroid response can be confirmed on clinical images (computed tomography, ultrasonography, endoscopic ultrasonography, magnetic resonance imaging, and 18F-fluorodeoxyglucose-positron emission tomography). Hence, the steroid response is included as an optional diagnostic item of AIP. Steroid treatment results in normalization of serological markers, including IgG4. Short- and long-term corticosteroid treatment may induce adverse events, including chronic glycometabolism, obesity, an immunocompromised status against infection, cataracts, glaucoma, osteoporosis, and myopathy. AIP is common in old age and is often associated with diabetes mellitus (33–78%). Thus, there is an argument for corticosteroid therapy in diabetes patients with no symptoms. With low-dose steroid treatment or treatment withdrawal, there is a high incidence of AIP recurrence (24–52%). Therefore, there is a need for long-term steroid maintenance therapy and/or steroid-sparing agents (immunomodulators and rituximab). Corticosteroids play a critical role in the diagnosis and treatment of AIP.

scholarly journals A Follow-Up Study of a European IgG4-Related Disease Cohort Treated with Rituximab

2021 ◽  
Vol 10 (6) ◽  
pp. 1329
Author(s):  
Johanna Backhus ◽  
Christian Neumann ◽  
Lukas Perkhofer ◽  
Lucas A Schulte ◽  
Benjamin Mayer ◽  
...  
Keyword(s):  
Disease Activity ◽  
Clinical Activity ◽  
Inflammatory Disorder ◽  
International Consensus ◽  
Steroid Pulse ◽  
Related Disease ◽  
Igg4 Related Disease

Objectives: IgG4-related disease (IgG4-RD) is a chronic fibro-inflammatory disorder affecting virtually any organ. Type 1 autoimmune (type 1 AIP) is its pancreatic manifestation. To date, steroids are considered the first-line pancreatitis treatment. The CD20-binding antibody rituximab (RTX) appears a promising steroid-sparing therapy, although long-term data are lacking. We aimed to bridge this gap with a cohort of IgG4-RD patients treated with RTX and to assess the potential value of the Responder Index (RI) as a discriminatory score for disease activity. Methods: We retrospectively evaluated 46 patients from a tertiary referral centre who were diagnosed with IgG4-RD and/or type 1 AIP according to the International Consensus Diagnostic Criteria or Unifying-AIP criteria between June 2006 and August 2019. Results: Patients resembled previous cohorts in terms of characteristics, diagnosis, and therapeutic response. Thirteen of the 46 patients with IgG4-RD/type 1 AIP were treated with RTX pulse therapy due to relapse, adverse reactions to steroids, or high-risk constellations predicting a severe course of disease with multi-organ involvement. Median follow-up after diagnosis was 52 months for all subjects, and 71 months in IgG4-RD patients treated with RTX. While patients in the RTX group showed no significant response to an initial steroid pulse, clinical activity as measured by the RI significantly decreased in the short-term after RTX induction. Within 16 months, 61% of patients relapsed in the RTX group but responded well to re-induction. Clinical and laboratory parameters improved equally in response to RTX. Conclusion: RTX therapy in patients with IgG4-RD is an effective and safe treatment to induce treatment response and possible long-term remission. Repeated RTX administration after 6–9 months may be of value in reducing the risk of relapse. The RI appears to be a reasonable index to assess disease activity and to identify patients with IgG4-related disease who may benefit from B-cell-depleting therapy.

scholarly journals PRM58 Long-Term Validation of the IMS CORE Diabetes Model in Type 1 and Type 2 Diabetes

2012 ◽  
Vol 15 (7) ◽  
pp. A470 ◽  
Author(s):  
V. Foos ◽  
J.L. Palmer ◽  
D. Grant ◽  
A. Lloyd ◽  
M. Lamotte ◽  
...  
Keyword(s):  
Type 2 Diabetes ◽  
Diabetes Model

scholarly journals Sex-Related Differences of Acute and Chronic Pancreatitis in Adults

2021 ◽  
Vol 10 (2) ◽  
pp. 300
Author(s):  
Madeline Drake ◽  
Shah-Jahan M. Dodwad ◽  
Joy Davis ◽  
Lillian S. Kao ◽  
Yanna Cao ◽  
...  
Keyword(s):  
Chronic Pancreatitis ◽  
The United States ◽  
Prevention Strategies ◽  
Endoscopic Retrograde ◽  
Effective Prevention ◽  
Type 1 Autoimmune Pancreatitis ◽  
Acute And Chronic Pancreatitis ◽  
Effective Prevention Strategies

The incidence of acute and chronic pancreatitis is increasing in the United States. Rates of acute pancreatitis (AP) are similar in both sexes, but chronic pancreatitis (CP) is more common in males. When stratified by etiology, women have higher rates of gallstone AP, while men have higher rates of alcohol- and tobacco-related AP and CP, hypercalcemic AP, hypertriglyceridemic AP, malignancy-related AP, and type 1 autoimmune pancreatitis (AIP). No significant sex-related differences have been reported in medication-induced AP or type 2 AIP. Whether post-endoscopic retrograde cholangiopancreatography pancreatitis is sex-associated remains controversial. Animal models have demonstrated sex-related differences in the rates of induction and severity of AP, CP, and AIP. Animal and human studies have suggested that a combination of risk factor profiles, as well as genes, may be responsible for the observed differences. More investigation into the sex-related differences of AP and CP is desired in order to improve clinical management by developing effective prevention strategies, diagnostics, and therapeutics.

Abstract 555: The Universal Clinical Classification of Acute Myocardial Infarction is a Better Predictor of Long Term Outcomes than ST Segment Classification

Circulation ◽  
2008 ◽  
Vol 118 (suppl_18) ◽  
Author(s):  
Manuel A Gonzalez ◽  
Dana Eilen ◽  
Rana A Marzouq ◽  
Saed Awadallah ◽  
Hiren R Patel ◽  
...  
Keyword(s):  
Risk Factors ◽  
Cardiovascular Risk ◽  
Inverse Relationship ◽  
Long Term Outcomes ◽  
St Segment ◽  
Vascular Beds

Introduction: The universal classification (UC) of AMI aims to facilitate cross-study analysis, yet the long-term outcomes using UC are largely unknown. Hypothesis: We tested the hypothesis that the long-term outcome of patients with AMI is better predicted by UC than ST segment classification. Methods: We conducted a prospective study of 348 consecutive patients with AMI with mean follow-up of 30.6 months. The primary outcome was the major adverse cardiovascular events (MACE) [composite of all causes of mortality, recurrent AMI, and stroke]. Multivariate and survival analysis of MACE was performed. Results: The study population was STEMI=168, NSTEMI=180, Type 1=278, Type 2=55, Type 3=5, Type 4a=2, Type 4b=5, and Type 5=3. During follow-up 80 patients died, 31 had an AMI, and 7 had a stroke. UC correlates with the ST segment classification (p<0.005). MACE free survival was different for Type 1 and Type 2 (p=0.043), but not for STEMI and NSTEMI. There was a positive association between MACE and the quartile of peak Troponin, number of cardiovascular risk factors, and number of vascular beds affected, and an inverse relationship with the utilization of discharge cardiovascular protective medications (all p≤0.01). No such inverse relationship existed for Type 2. Conclusions: UC of AMI is a better long-term predictor of MACE. The quartile of peak Troponin levels, cardiovascular risk factors, and number of vascular beds affected are independent predictors of MACE, while cardiac medications protect against MACE, except in Type 2 patients.

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