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.

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 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.

Autoimmune pancreatitis associated with primary sclerosing cholangitis: MR imaging findings

2001 ◽  
Vol 11 (8) ◽  
pp. 1401-1404 ◽  
Author(s):  
Isabel Eerens ◽  
Dirk Vanbeckevoort ◽  
Werner Vansteenbergen ◽  
Lieven Van Hoe
Keyword(s):  
Mr Imaging ◽  
Primary Sclerosing Cholangitis ◽  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Imaging Findings

scholarly journals Kidney Involvement in Patients with Type 1 Autoimmune Pancreatitis

2019 ◽  
Vol 8 (2) ◽  
pp. 258
Author(s):  
Miroslav Vujasinovic ◽  
Raffaella Pozzi Mucelli ◽  
Roberto Valente ◽  
Caroline Verbeke ◽  
Stephan Haas ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Lymphocytic Infiltration ◽  
Normal Serum ◽  
International Consensus ◽  
Kidney Involvement ◽  
Type 1 Autoimmune Pancreatitis ◽  
Elevated Creatinine ◽  
Imaging Signs

Introduction: Autoimmune pancreatitis (AIP) type 1 is a special form of chronic pancreatitis with a strong lymphocytic infiltration as the pathological hallmark and other organ involvement (OOI). IgG4-related kidney disease (IgG4-RKD) was first reported as an extrapancreatic manifestation of AIP in 2004. The aim of the present study was to determine the frequency and clinical impact of kidney lesions observed in patients with AIP type 1. Methods: We performed a single-centre retrospective study on a prospectively collected cohort of patients with a histologically proven or highly probable diagnosis of AIP according to the International Consensus Diagnostic Criteria (ICDC) classification. Results: Seventy-one patients with AIP were evaluated. AIP type 1 was diagnosed in 62 (87%) patients. Kidney involvement was present in 17 (27.4%) patients with AIP type 1: 15 (88.2%) males and 2 (11.8%) females. Laboratory and/or imaging signs of kidney involvement were presented at the time of AIP diagnosis in eight (47.1%) patients. In other patients, the onset of kidney involvement occurred between four months and eight years following diagnosis. At the time of the diagnosis of kidney involvement, eight (47.1%) patients showed elevated creatinine, and nine (52.9%) patients showed normal serum creatinine. None of the patients were treated with dialysis. Conclusions: IgG4-RKD was present in 27.4% of patients with AIP type 1, with male gender predominance. In cases of early diagnosis and cortisone treatment, the clinical course was mild in most cases. Regular laboratory control of renal function should be a part of the follow-up of patients with AIP type 1.

scholarly journals Primary sclerosing cholangitis with increased immunoglobulin G4 levels

Medicine ◽  
2019 ◽  
Vol 98 (50) ◽  
pp. e18411
Author(s):  
Qing-Ling Chen ◽  
Rui Zhong ◽  
Xiao-Xue Zhang ◽  
Li-Na Feng ◽  
Xiao-Yu Wen ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Immunoglobulin G4

Are tumefactive lesions classified as sclerosing mesenteritis a subset of IgG4-related sclerosing disorders?

2008 ◽  
Vol 61 (10) ◽  
pp. 1093-1097 ◽  
Author(s):  
T S Chen ◽  
E A Montgomery
Keyword(s):  
High Power ◽  
Autoimmune Pancreatitis ◽  
Plasma Cells ◽  
Relative Proportion ◽  
Sclerosing Mesenteritis ◽  
Fat Necrosis ◽  
Lymphoplasmacytic Sclerosing Pancreatitis ◽  
Reactive Plasma ◽  
Sclerosing Pancreatitis ◽  
The Relationship

Background:The relationship between tumefactive lesions classified as sclerosing mesenteritis and IgG4-related sclerosing disorders (eg, lymphoplasmacytic sclerosing pancreatitis/autoimmune pancreatitis) remains uncertain.Aims:To review lesions coded as “sclerosing mesenteritis” for findings in keeping with IgG4-related sclerosing disorders.Methods:Inclusion in the study required available paraffin blocks for IgG4 staining and documentation of a mass lesion.Results:A total of nine mesenteric lesions (3–14 cm) were identified in 6 male and 3 female patients. On H&E-stained sections, all were characterised as loosely marginated fibroinflammatory processes with variable amounts of fat necrosis. Lymphocytic venulitis/phlebitis was identified in 8 of 9 cases. IgG and IgG4 expression in lesional plasma cells was assessed by immunohistochemistry. IgG4-positive plasma cells were counted in the areas of greatest density in ⩾3 high power fields (HPFs). The highest number per HPF was recorded and a score assigned based on the following scale: <5/HPF, none/minimal; 5–10/HPF, mild; 11–30/HPF, moderate; >30/HPF, marked. The relative proportion of IgG4-reactive plasma cells to total IgG-positive plasma cells was assessed. IgG4-reactive plasma cells ranged from 0 to >100 in the most dense zones (3 cases, none/minimal; 4 cases, moderate; 2 cases, marked).Conclusions:Although this study is limited by small numbers, findings suggest that some tumefactive lesions regarded as sclerosing mesenteritis may be a subset of IgG4-related sclerosing disorders.

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