Mo1383 IGG4-NEGATIVE SCLEROSING PANCREATITIS: A THIRD SUBTYPE OF AUTOIMMUNE PANCREATITIS?

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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LYMPHOPLASMACYTIC SCLEROSING PANCREATITIS (LPSP) AND IDIOPATHIC DUCT CENTRIC PANCREATIS (IDCP) ARE DISTINCT CLINICAL FORMS OF AUTOIMMUNE PANCREATITIS (AIP)

Pancreas ◽

10.1097/01.mpa.0000335394.75884.ea ◽

2008 ◽

Vol 37 (4) ◽

pp. 497 ◽

Cited By ~ 7

Author(s):

A. Sugumar ◽

T. C. Smyrk ◽

N Takahashi ◽

M. J. Levy ◽

S. T. Chari

Keyword(s):

Autoimmune Pancreatitis ◽

Lymphoplasmacytic Sclerosing Pancreatitis ◽

Clinical Forms ◽

Sclerosing Pancreatitis

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Autoimmune Pancreatitis with Histologically Proven Lymphoplasmacytic Sclerosing Pancreatitis with Granulocytic Epithelial Lesions

Internal Medicine ◽

10.2169/internalmedicine.51.6859 ◽

2012 ◽

Vol 51 (7) ◽

pp. 733-737 ◽

Cited By ~ 8

Author(s):

Tsukasa Ikeura ◽

Makoto Takaoka ◽

Kazushige Uchida ◽

Masaaki Shimatani ◽

Hideaki Miyoshi ◽

...

Keyword(s):

Autoimmune Pancreatitis ◽

Lymphoplasmacytic Sclerosing Pancreatitis ◽

Sclerosing Pancreatitis ◽

Epithelial Lesions

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Clinical and Immunopathologic Profile of Mexican Patients with IgG4 Autoimmune Pancreatitis

ISRN Rheumatology ◽

10.5402/2012/164914 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 2

Author(s):

María T. Bourlon ◽

Christianne Bourlon ◽

Yemil Atisha-Fregoso ◽

Fredy Chable-Montero ◽

Marco A. Teliz ◽

...

Keyword(s):

Weight Loss ◽

Autoimmune Pancreatitis ◽

Clinical Presentation ◽

Clinical Manifestations ◽

Serum Levels ◽

Current Data ◽

Histological Subtypes ◽

Sclerosing Pancreatitis ◽

The Mean ◽

Mexican Patients

Autoimmune pancreatitis is part of the spectrum of IgG4-associated diseases. Its diagnostic criteria and histological subtypes have been formally proposed recently and although based on current data it has been suggested that there are differences in clinical presentation among populations, more research is needed to properly establish if this heterogeneity exists. In this paper, we describe 15 cases of autoimmune pancreatitis diagnosed at a Mexican centre of reference, all of them associated to the lymphoplasmocytic sclerosing pancreatitis variant. The mean age at the onset of symptoms was 47.5 ± 14.4 years, and 53% of patients were male. The main manifestations were weight loss (87%), obstructive jaundice (53%), and acute (27%) and chronic (27%) pancreatitis. Only 20% of patients had high IgG4 serum levels at the time of diagnosis. All patients receiving prednisone responded favourably, both in their pancreatic and extrapancreatic manifestations. Clinical manifestations of Mexican patients showed certain differences with respect to those usually reported.

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

United European Gastroenterology Journal ◽

10.1177/2050640620916027 ◽

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.

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

Case Reports in Gastroenterology ◽

10.1159/000486443 ◽

2018 ◽

Vol 12 (1) ◽

pp. 46-55 ◽

Cited By ~ 2

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.

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Fine Needle Aspirate of Autoimmune Pancreatitis (Lymphoplasmacytic Sclerosing Pancreatitis): Cytomorphologic Characteristics and Clinical Correlates

Acta Cytologica ◽

10.1159/000336135 ◽

2012 ◽

Vol 56 (3) ◽

pp. 228-232 ◽

Cited By ~ 16

Author(s):

Brittany J. Holmes ◽

Ralph H. Hruban ◽

Christopher L. Wolfgang ◽

Syed Z. Ali

Keyword(s):

Autoimmune Pancreatitis ◽

Fine Needle Aspirate ◽

Fine Needle ◽

Lymphoplasmacytic Sclerosing Pancreatitis ◽

Clinical Correlates ◽

Sclerosing Pancreatitis

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Management of autoimmune pancreatitis

Herald of Pancreatic Club ◽

10.33149/vkp.2020.03.02 ◽

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).

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

Gastroenterology Research and Practice ◽

10.1155/2017/3246459 ◽

2017 ◽

Vol 2017 ◽

pp. 1-11 ◽

Cited By ~ 10

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.

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