scholarly journals Cardiovascular and Lung Involvement in Patients with Autoimmune Pancreatitis

2020 ◽  
Vol 9 (2) ◽  
pp. 409 ◽  
Author(s):  
Sara Nikolic ◽  
Katharina Brehmer ◽  
Nikola Panic ◽  
Roberto Valente ◽  
J.-Matthias Löhr ◽  
...  
Keyword(s):  
Clinical Data ◽  
Autoimmune Pancreatitis ◽  
University Hospital ◽  
Vascular Involvement ◽  
Lung Involvement ◽  
Immunoglobulin G4 ◽  
Historical Cohort ◽  
Cardiovascular Involvement ◽  
Sclerosing Pancreatitis

Introduction: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immune-mediated disease characterised pathologically by the infiltration of IgG4-bearing plasma cells into the involved organs. Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis with a heavy lymphocytic infiltration and two distinct histopathological subtypes, namely: lymphoplasmacytic sclerosing pancreatitis (AIP type 1) and idiopathic duct-centric pancreatitis (AIP type 2). Lung involvement and aortic involvement have been reported in 12% and 9% of patients with systemic IgG4-RD, respectively. In series including patients with AIP, both lung and aortic involvement were described in 2% of the patients. Most of the epidemiological data come from Japan, and there is a lack of information from Europe, especially the Scandinavian countries. Patients and methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients diagnosed with AIP at the Department for Digestive Diseases at Karolinska University Hospital in Stockholm, Sweden, from 2004 to 2019. Demographic and clinical data were collected from the medical charts. Results: One hundred and thirty-three patients with AIP were analysed. Six patients were excluded because they lacked some of the clinical data relevant to the study. Demographic and clinical features of 127 patients were presented. There were 98 patients with AIP type 1-35 (35.7%) female and 63 (64.3%) male, with a mean age of 55.4 ± 18.2. Among them, 15 (15.3%) patients had lung and/or cardiovascular involvement-11 (11.2%) patients had lung involvement, 10 (10.2%) patients had cardiovascular involvement (six patients had both). Most of them (67.0%) had never smoked. The mean follow-up time of the patients with AIP type 1 was 49 months. Conclusions: Lung and/or cardiovascular involvement were diagnosed in 15 (15.3%) patients in our historical cohort of patients with AIP type 1. Most of the lung involvement was presented in the form of nodular lesions in the lungs, non-specific infiltrates, “ground-glass” appearance with pleura thickening, and effusion. Aortic involvement was a major form of vascular involvement in patients with AIP, as in previous published studies on patients with IgG4-RD.

scholarly journals Immunoglobulin G subtypes-1 and 2 differentiate immunoglobulin G4-associated sclerosing cholangitis from primary sclerosing cholangitis

2020 ◽  
Vol 8 (5) ◽  
pp. 584-593
Author(s):  
Miroslav Vujasinovic ◽  
Pia Maier ◽  
Hartwig Maetzel ◽  
Roberto Valente ◽  
Raffaella Pozzi-Mucelli ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
High Specificity ◽  
Lymphocytic Infiltration ◽  
Control Group ◽  
Immunoglobulin G4 ◽  
Lymphoplasmacytic Sclerosing Pancreatitis ◽  
Sclerosing Pancreatitis

Background Autoimmune pancreatitis is a special form of chronic pancreatitis with strong lymphocytic infiltration and two histopathological distinct subtypes, a lymphoplasmacytic sclerosing pancreatitis and idiopathic duct centric pancreatitis. Immunoglobulin G4-associated cholangitis may be present at the time of autoimmune pancreatitis type 1 diagnosis or occur later over the course of the disease. Immunoglobulin G4 is considered reliable but not an ideal marker for diagnosis of autoimmune pancreatitis type 1 with reported sensitivity between 71–81%. It is essential to differentiate sclerosing cholangitis with autoimmune pancreatitis from primary sclerosing cholangitis as the treatment and prognosis of the two diseases are totally different. It was the aim of the study to find a marker for immunoglobulin G4-associated cholangitis that would distinguish it from primary sclerosing cholangitis. Patients and methods We performed a retrospective analysis of patients with autoimmune pancreatitis at our outpatient clinic. Patients from the primary sclerosing cholangitis registry were taken as a control group. Blood samples for the measurement of immunoglobulin subclasses were analysed at the time of diagnosis. Results Patients with autoimmune pancreatitis and immunoglobulin G4-associated cholangitis had higher values of immunoglobulin G2 when compared to autoimmune pancreatitis alone or primary sclerosing cholangitis with a high specificity (97%) and high positive predictive value (91%). In patients with normal or low immunoglobulin G2 or immunoglobulin G4, a high level of immunoglobulin G1 indicated primary sclerosing cholangitis. Conclusion Immunoglobulin G1 and immunoglobulin G2 can distinguish patients with immunoglobulin G4-associated cholangitis from those with primary sclerosing cholangitis.

Surgery in autoimmune pancreatitis

2021 ◽  
Author(s):  
Sara Nikolic ◽  
Poya Ghorbani ◽  
Raffaella Pozzi Mucelli ◽  
Sam Ghazi ◽  
Francisco Baldaque- Silva ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
University Hospital ◽  
Premalignant Lesions ◽  
Single Centre ◽  
Age At Surgery ◽  
Diagnostic Work ◽  
Work Up

Introduction: Autoimmune pancreatitis (AIP) is a disease that may mimic malignant pancreatic lesions both in terms of symptomatology and imaging appearance. The aim of the present study is to analyse experiences of surgery in patients with AIP in one of the largest European cohorts. Methods: We performed a single-centre retrospective study of patients diagnosed with AIP at the Department of Abdominal Diseases at Karolinska University Hospital in Stockholm, Sweden, between January 2001 and October 2020. Results: There were 159 patients diagnosed with AIP, and among them 35 (22.0%) patients had surgery: 20 (57.1%) males and 15 (42.9%) females; average age at surgery was 59 years (range 37-81). Follow-up period after surgery was 67 months (range 1-235). AIP type 1 was diagnosed in 28 (80%) patients and AIP type 2 in 7 (20%) patients. Malignant and premalignant lesions were diagnosed in 8 (22.9%) patients for whom AIP was not the primary differential diagnosis but, in all cases, it was described as a simultaneous finding and recorded in retrospective analysis in histological reports of surgical specimens. Conclusions: Diagnosis of AIP is not always straightforward, and, in some cases, it is not easy to differentiate it from the malignancy. Surgery is generally not indicated for AIP but might be considered in patients when suspicion of malignant/premalignant lesions cannot be excluded after complete diagnostic work-up.

scholarly journals A Rare Multifocal Pattern of Type 2 Autoimmune Pancreatitis with Negative IgG4: A Potential Diagnostic Pitfall That May Mimic Multifocal Pancreatic Adenocarcinoma

2018 ◽  
Vol 12 (1) ◽  
pp. 46-55 ◽  
Author(s):  
Partha Hota ◽  
Tejas Patel ◽  
Xiaofeng Zhao ◽  
Nirag Jhala ◽  
Omar Agosto
Keyword(s):  
Acute Pancreatitis ◽  
Mr Imaging ◽  
Pancreatic Adenocarcinoma ◽  
Autoimmune Pancreatitis ◽  
Diagnostic Pitfall ◽  
First Case ◽  
Imaging Characteristics ◽  
Sclerosing Pancreatitis

Autoimmune pancreatitis (AIP) is an increasingly recognized form of acute pancreatitis characterized by obstructive jaundice with a rapid and dramatic treatment response to steroid therapy. Recently, AIP has been divided into two distinct phenotypes: lymphoplasmocytic sclerosing pancreatitis AIP (type 1) and idiopathic duct-centric pancreatitis AIP (type 2); each of which have their own distinct demographics, diagnostic criteria, and histopathological features. We report, to the best of our knowledge, the first case of a multifocal pattern of type 2 AIP characterized with both CT and MR imaging. This rare imaging pattern of AIP may mimic the appearance of more worrisome malignant etiologies such as multifocal pancreatic adenocarcinoma or lymphoma, with overlapping imaging characteristics potentially complicating or delaying diagnosis. Therefore, recognition of this atypical pattern of AIP and avoidance of this potential diagnostic pitfall is crucial.

scholarly journals Peripancreatic vascular involvement in patients with type 1 autoimmune pancreatitis

2021 ◽  
Vol 0 (0) ◽  
pp. 0-0
Author(s):  
Meizi Li ◽  
Xiaoyin Bai ◽  
Kai Xu ◽  
Xi Wu ◽  
Tao Guo ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Vascular Involvement ◽  
Type 1 Autoimmune Pancreatitis

scholarly journals Management of autoimmune pancreatitis

2020 ◽  
Vol 48 (3) ◽  
pp. 13-17
Author(s):  
V. S. Akshintala ◽  
V. K. Singh
Keyword(s):  
Autoimmune Pancreatitis ◽  
Pancreatic Disease ◽  
Lymphoplasmacytic Sclerosing Pancreatitis ◽  
Sclerosing Pancreatitis ◽  
Histological Characteristics ◽  
Igg4 Level

The article contains modern data on such a chronic pancreatic disease as autoimmune pancreatitis (AIP). Statistical data on the prevalence and incidence of AIP are presented, the current international classification of this pathology is considered, the main features of type 1 AIP (lymphoplasmacytic sclerosing pancreatitis, LPSP) and type 2 AIP (idiopathic duct centric pancreatitis, IDCP) are put forward. The clinical manifestation of these types is different: obstructive jaundice develops more often in patients with LPSP, while patients with IDCP mostly have acute pancreatitis. The presence and variety of extrapancreatic manifestations of the disease with extremely frequent concomitant development of ulcerative colitis is emphasized. The features of serological diagnosis of AIP and the role of IgG4 level in determining the type of disease are considered. The proper techniques of AIP instrumental diagnostics are listed, the typical changes detected during computed tomography and the distinctive histological characteristics of LPSP and IDCP are considered. The feasibility of a differential diagnosis between AIP and pancreatic cancer is indicated by analyzing the results of serological, imaging, and histological studies. The algorithm of management of AIP patients depending on the type of disease is described, as well as the tactics of prescribing corticosteroids, immunomodulators upon LPSP and IDCP. Immunomodulators of choice (mycophenolate mofetil, azathioprine) are indicated, and rituximab administration features are considered. Possible signs of AI recurrence are listed (IgG4-sclerosing cholangitis, high IgE level).

scholarly journals From Pathogenesis, Clinical Manifestation, and Diagnosis to Treatment: An Overview on Autoimmune Pancreatitis

2017 ◽  
Vol 2017 ◽  
pp. 1-11 ◽  
Author(s):  
Ou Cai ◽  
Shiyun Tan
Keyword(s):  
Autoimmune Pancreatitis ◽  
Clinical Manifestations ◽  
Type I ◽  
International Consensus ◽  
Glucocorticoid Treatment ◽  
Immunoglobulin G4 ◽  
Lymphoplasmacytic Sclerosing Pancreatitis ◽  
Serum Igg4 ◽  
Sclerosing Pancreatitis ◽  
Upper Abdominal

Autoimmune pancreatitis (AIP) is a special type of chronic pancreatitis which is autoimmune mediated. The international consensus diagnostic criteria (ICDC) 2011 proposed two types of AIP: type I is associated with histological pattern of lymphoplasmacytic sclerosing pancreatitis (LPSP), characterized by serum IgG4 elevation, whereas type 2 is named idiopathic duct-centric pancreatitis (IDCP), with granulocytic epithelial lesion (GEL) and immunoglobulin G4 (IgG4) negative. The pathogenic mechanism is unclear now; based on genetic factors, disease specific or related antigens, innate and adaptive immunity may be involved. The most common clinical manifestations of AIP are obstructive jaundice and upper abdominal pain. The diagnosis can be made by a combination of parenchymal and ductal imaging, serum IgG4 concentrations, pancreatic histology, extrapancreatic disease, and glucocorticoid responsiveness according to ICDC 2011. Because of the clinical and imaging similarities with pancreatic cancer, general work-up should be done carefully to exclude pancreatic malignant tumor before empirical trial of glucocorticoid treatment. Glucocorticoid is the most common drug for AIP to induce remission, while there still exists controversy on steroid maintenance and treatment for relapse. Further studies should be done to identify more specific serum biomarkers for AIP, the pathogenic mechanisms, and the treatment for relapse.

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 Outcome and Genetic Factors in IgG4-Associated Autoimmune Pancreatitis and Cholangitis: A Single Center Experience

2017 ◽  
Vol 2017 ◽  
pp. 1-9 ◽  
Author(s):  
Matthias Buechter ◽  
Paul Manka ◽  
Falko Markus Heinemann ◽  
Monika Lindemann ◽  
Benjamin Juntermanns ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Genetic Factors ◽  
Statistical Significance ◽  
Female Gender ◽  
Asian Studies ◽  
Immunoglobulin G4 ◽  
Equal Distribution ◽  
Single Center Experience

Introduction. Most investigations on autoimmune pancreatitis (AIP) were published on Asian cohorts while those on Caucasians are limited. However, there might be differences related to the origin.Patients and Methods. We analyzed 36 patients and compared type 1 (AIP1) with type 2 (AIP2).Results. The majority of patients suffered from AIP1 (55.6%). AIP1 patients were significantly older than AIP2 patients (54.4 versus 40.8 years). Moreover, 85.0% of AIP1 patients had concurrent autoimmune cholangitis (AIC) while 18.8% of AIP2 patients suffered from overlap to ulcerative colitis (UC). However, AIP1 patients revealed a cholestatic course and had significantly higher immunoglobulin G4 levels (IgG4). When compared to allele frequencies in healthy controls, in patients with AIP1 HLA-B8 reached statistical significance. Response to steroids was excellent in both groups, but we noticed high rates of relapse especially in AIP1 patients. Finally, 3 patients with AIP1 were diagnosed with cholangiocellular carcinoma (CCC).Conclusion. In contrast to Asian studies, we found an almost equal distribution of AIP1 and AIP2 patients in our German cohort. AIP2 patients were younger and mostly of female gender whereas AIP1 patients revealed higher IgG4 levels and involvement of the biliary tract in sense of IgG4-associated cholangitis.

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