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

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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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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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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