scholarly journals Autoimmune Pancreatitis Type 1 Associated with a Pancreatic Pseudocyst

2019 ◽  
Vol 13 (1) ◽  
pp. 195-199
Author(s):  
Felix Hesse ◽  
Rickmer Braren ◽  
Roland M. Schmid ◽  
Veit Phillip
Keyword(s):  
Differential Diagnosis ◽  
Autoimmune Pancreatitis ◽  
Pancreatic Pseudocyst ◽  
Elevated Serum ◽  
Response To Treatment ◽  
Cystic Lesions ◽  
Serum Igg4 ◽  
Underlying Pathology ◽  
Tomography Scan

Pancreatic cystic lesions comprise diverse entities with different histopathological characteristics. Differential diagnosis is often challenging. Autoimmune pancreatitis (AIP) is usually not considered an underlying pathology in the differential diagnosis of peri-/pancreatic pseudo-/cystic lesions. We report the case of a 73-year-old male with diffuse pancreatic enlargement and an adjacent cystic lesion (60 × 80 mm) on computed tomography scan. Based on these imaging findings and an elevated serum IgG4 concentration, AIP complicated by a pancreatic pseudocyst was diagnosed, and treatment with glucocorticoids was started. Regular follow-ups showed a good response to treatment with regression of the pancreatic pseudocyst and remittent pancreatic swelling.

scholarly journals Identification of serum IFN-α and IL-33 as novel biomarkers for type 1 autoimmune pancreatitis and IgG4-related disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Kosuke Minaga ◽  
Tomohiro Watanabe ◽  
Akane Hara ◽  
Ken Kamata ◽  
Shunsuke Omoto ◽  
...  
Keyword(s):  
Autoimmune Pancreatitis ◽  
Characteristic Curve ◽  
Elevated Serum ◽  
Normal Serum ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Number Of Patients ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

Abstract IgG4-related disease (IgG4-RD) is a multi-organ autoimmune disease characterized by elevated serum IgG4 concentration. Although serum IgG4 concentration is widely used as a biomarker for IgG4-RD and type 1 autoimmune pancreatitis (AIP), a pancreatic manifestation of IgG4-RD, a significant number of patients have normal serum IgG4 levels, even in the active phase of the disease. Recently, we reported that the development of experimental AIP and human type 1 AIP is associated with increased expression of IFN-α and IL-33 in the pancreas. In this study, we assessed the utility of serum IFN-α and IL-33 levels as biomarkers for type 1 AIP and IgG4-RD. Serum IFN-α and IL-33 concentrations in patients who met the diagnostic criteria for definite type 1 AIP and/or IgG4-RD were significantly higher than in those with chronic pancreatitis or in healthy controls. Strong correlations between serum IFN-α, IL-33, and IgG4 concentrations were observed. Diagnostic performance of serum IFN-α and IL-33 concentrations as markers of type 1 AIP and/or IgG4-RD was comparable to that of serum IgG4 concentration, as calculated by the receiver operating characteristic curve analysis. Induction of remission by prednisolone treatment markedly decreased the serum concentration of these cytokines. We conclude that serum IFN-α and IL-33 concentrations can be useful as biomarkers for type 1 AIP and IgG4-RD.

scholarly journals IgG4-Seronegative Autoimmune Pancreatitis and Sclerosing Cholangitis

2015 ◽  
Vol 2015 ◽  
pp. 1-6
Author(s):  
Allon Kahn ◽  
Anitha D. Yadav ◽  
M. Edwyn Harrison
Keyword(s):  
Autoimmune Pancreatitis ◽  
Sclerosing Cholangitis ◽  
Clinical Presentation ◽  
Surgical Intervention ◽  
Comprehensive Evaluation ◽  
Elevated Serum ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Type 1 Autoimmune Pancreatitis

IgG4-related disease is a relatively novel clinical entity whose gastrointestinal manifestations include type 1 autoimmune pancreatitis (AIP) and IgG4-associated sclerosing cholangitis. The presence of elevated serum IgG4 is suggestive but not essential for the diagnosis of type 1 AIP and is a pervasive feature of the proposed diagnostic criteria. The differential diagnosis of type 1 AIP includes malignant conditions, emphasizing the importance of a deliberate, comprehensive evaluation. Management of patients with a suggestive clinical presentation, but without serum IgG4 elevation, is difficult. Here we present three cases of IgG4-seronegative AIP and sclerosing cholangitis that responded to empiric steroid therapy and discuss approach considerations. These cases demonstrate the value of meticulous application of existing diagnostic algorithms to achieve a clinical diagnosis and avoid surgical intervention.

scholarly journals Pancreatic Solid Focal Lesions: Differential Diagnosis between Autoimmune Pancreatitis and Pancreatic Cancer

2019 ◽  
Vol 37 (5) ◽  
pp. 416-421 ◽  
Author(s):  
Petr Dite ◽  
Ivo Novotny ◽  
Jana Dvorackova ◽  
Bohuslav Kianicka ◽  
Martin Blaho ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Differential Diagnosis ◽  
Autoimmune Pancreatitis ◽  
Diagnostic Features ◽  
Pancreatic Lesion ◽  
Immunoglobulin G4 ◽  
Normal Value ◽  
Serum Igg4 ◽  
Igg4 Level

Background: Diagnosis of pancreatic cancer (PC) in early stages is still challenging for gastroenterologists. The early detection of cancer is one of the utmost importance for the successful therapy of this malignancy. An accurate differential diagnosis of focal pancreatic lesions plays also an important role, whether it is differential diagnosis of chronic pancreatitis from PC or autoimmune pancreatitis (AIP) from PC. Raised serum immunoglobulin G4 (IgG4) levels to twice the normal value are considered one of significant diagnostic features of type 1 AIP. However, IgG4 can be increased also in patients with PC, but levels usually do not exceed twice the normal value. Methods: In years 2012–2017, IgG4 serum levels were examined in 115 patients with histologically confirmed PC. Patients with PC and elevated IgG4 level (above 135 mg/dL) had tested their histological resection specimens or bioptic specimens from pancreatic lesion, with targeted detection of the presence of IgG4 and plasmocytes in the pancreatic tissue and changes characteristic for type 1 AIP. Results: A plasmatic IgG4 level in 115 patients with diagnosed PC was higher than 135 mg/dL in 14 patients (12.2%). Out of them, 2 patients (1.7%) revealed a serum IgG4 level higher than double the normal value, that is, higher than 270.0 mg/dL (suggestive of AIP). One patient met histological criteria for diagnosis of AIP in the simultaneous presence of PC. Conclusion: Diagnosis of early cancer stages, particularly differentiating AIP from PC can be sometimes problematic. IgG4 levels can be slightly elevated also in case of PC. A targeted biopsy of the pancreas is the method of choice in cases suspected from a focal form of AIP and we recommend to prefer it over other modalities, such as, for example, response to steroid therapy.

The coexistence of autoimmune pancreatitis and elevated serum IGG4 in autoimmune diseases

2011 ◽  
Vol 49 (05) ◽  
Author(s):  
V Terzin ◽  
I Földesi ◽  
L Kovács ◽  
G Pokornyi ◽  
T Wittmann ◽  
...  
Keyword(s):  
Autoimmune Diseases ◽  
Autoimmune Pancreatitis ◽  
Elevated Serum ◽  
Serum Igg4

scholarly journals Localized autoimmune pancreatitis mimicking pancreatic cancer: Case report and literature review

2018 ◽  
Vol 46 (4) ◽  
pp. 1657-1665 ◽  
Author(s):  
Wen-Ling Hsu ◽  
Shu-Min Chang ◽  
Pei-Yin Wu ◽  
Chin-Chuan Chang
Keyword(s):  
Pancreatic Cancer ◽  
Autoimmune Pancreatitis ◽  
Elevated Serum ◽  
Pancreatic Head ◽  
Corticosteroid Treatment ◽  
Cancer Case ◽  
Pancreatic Lesion ◽  
Immunoglobulin G4 ◽  
Positron Emission ◽  
Serum Igg4

Autoimmune pancreatitis (AP) is a rare autoimmune pancreatic manifestation of systemic immunoglobulin G4 (IgG4)-related sclerosing disease. Distinguishing between AP and pancreatic cancer is crucial because the clinical courses, treatments, and prognoses of these two disease entities are quite different. We herein report a case involving a 52-year-old man with subacute epigastralgia who visited our hospital for evaluation of a suspicious pancreatic mass found during esophagogastroduodenoscopy. Enhanced computed tomography (CT) revealed an enlarged lesion in the pancreatic head with encasement of hepatic vessels. The lesion also exhibited increased 18F-fluorodeoxyglucose accumulation on positron emission tomography/CT imaging, which was highly suggestive of pancreatic cancer. After open biopsy, morphologic examination showed an inflammatory infiltrate in the pancreas, which was compatible with chronic sclerotic pancreatitis. Further laboratory tests revealed an elevated serum IgG4 level, and the diagnosis of sclerotic pancreatitis was then confirmed. After corticosteroid treatment, the pancreatic lesion showed shrinkage on follow-up CT, and the serum IgG4 titer decreased to the normal range. This case suggests that clinicians should be familiar with the clinical presentations and diagnostic criteria of AP versus pancreatic cancer. An awareness of the differences between these diseases may avoid misdiagnosis and unnecessary surgical intervention.

scholarly journals Multifocal Mass Lesions in Autoimmune Pancreatitis

2017 ◽  
Vol 11 (3) ◽  
pp. 686-693 ◽  
Author(s):  
Kazuhiro Suzumura ◽  
Etsuro Hatano ◽  
Naoki Uyama ◽  
Toshihiro Okada ◽  
Yasukane Asano ◽  
...  
Keyword(s):  
Magnetic Resonance ◽  
Autoimmune Pancreatitis ◽  
Malignant Tumors ◽  
Histopathological Examination ◽  
Elevated Serum ◽  
Pancreatic Head ◽  
Pancreatic Ducts ◽  
Diffuse Infiltration ◽  
Serum Igg4 ◽  
Mass Lesions

A 59-year-old male patient with jaundice was referred to our hospital because of mass lesions in the pancreatic head and tail. An immunological examination revealed an elevated serum IgG4 level. Computed tomography showed two clear boundary mass lesions in the pancreatic head and tail. Magnetic resonance imaging showed that the mass lesions exhibited low intensity on T1-weighted images and iso-intensity on T2-weighted images. Magnetic resonance cholangiopancreatography showed an obstruction of the main pancreatic duct in the pancreatic head and tail. The possibility of malignant tumors could not be ruled out; therefore, we performed total pancreatectomy. A histopathological examination of the nodular lesions revealed severe lymphoplasmacytic infiltration and inflammatory change around the pancreatic ducts. Immunohistochemistry revealed diffuse infiltration of IgG4-positive plasma cells in the nodules. According to these pathological findings, we diagnosed the patient with IgG4-related multifocal mass lesions of autoimmune pancreatitis (AIP). It is difficult to distinguish between focal type AIP and pancreatic cancer. We herein report a rare case of multifocal mass lesions in AIP and include bibliographical comments.

scholarly journals Clinical and morphological characteristics of type 1 and 2 autoimmune pancreatitis

2018 ◽  
Vol 46 (4) ◽  
pp. 330-337
Author(s):  
S. V. Lishchuk ◽  
Е. A. Dubova ◽  
K. А. Pavlov ◽  
Yu. D. Udalov
Keyword(s):  
Differential Diagnosis ◽  
Autoimmune Pancreatitis ◽  
Morphological Characteristics ◽  
High Power Field ◽  
Obliterative Phlebitis ◽  
Inflammatory Infiltration ◽  
Diagnostic Potential ◽  
The Mean

Rationale: In the recent years, an increased interest to autoimmune pancreatitis (AIP) has been seen, related to growing diagnostic potential. In its turn, this leads to an increase in numbers of diagnosed AIP cases. At present, two types of AIP have been described with diverse clinical manifestation and morphology of the pancreas. However, the reproducibility of the differential diagnosis between AIP type 1 and 2 is low even among pancreatic pathologists.Aim: To identify criteria for the morphologic diagnosis of AIP type 1 and 2.Materials and methods: A morphological study of biopsy and surgical specimens from 26 patients with AIP was performed. There were 22 cases of AIP type 1 and 4 cases of AIP type 2. In addition to hematoxylin eosin staining of the specimens, immunohistochemistry was used with counting of CD138+ absolute numbers, determination of IgG+ and IgG4+ cells in the inflammatory infiltrates, as well as the ratios of IgG4+/IgG+ and IgG4+/CD138+ cells.Results: AIP type 1 was characterized by storiform fibrosis of the pancreatic tissue (81.8% cases), involving the parapancreatic fat tissue, by moderateto-severe lymphoplasmocytic infiltration and signs of obliterative/non-obliterative phlebitis. Type 2 AIP was characterized by severe fibrosis with predominantly periductal (centrilobular) fibrosis and mild chronic inflammatory infiltration of the pancreas, while there was no extension of fibrosis and inflammatory infiltration to the parapancreatic tissues in any case. The mean number of CD138+ cell in AIP type 1 was 101.2 ± 27.9 per 1 high-power field (HPF), and in AIP type 2, it was 42.8 ± 20.9 per 1 HPF. The mean absolute number of IgG+ cells in AIP type 1 was 99.6 ± 25.7 per 1 HPF, whereas in AIP type 2, 42.1 ± 20.8 per 1 HPF. In AIP type 1, the mean number of IgG4+ plasmatic cells in the infiltrates was 74.5 ± 27.2 per 1 HPF, whereas in AIP type 2, it was 3.4 ± 2.7 per 1 HPF. The IgG4+/IgG+ ratio was 75 ± 12.6% vs. 8.4 ± 6.2%, and the IgG4+/CD138+ ratio was 72.4 ± 12.3% vs. 8.3 ± 5.9% in AIP type 1 and type 2, respectively.Conclusion: For the differential diagnosis of type 1 and 2 AIP, it is necessary to take into consideration not only typical histological abnormalities, but also the numbers of CD138+, IgG+ and IgG4+ cells within the inflammatory infiltrate, as well as the IgG4+/IgG+ and IgG4+/CD138+ ratios.

scholarly journals [Eosinophilic pancreatitis and autoimmune pancreatitis: comparison, differential diagnosis, and treatment]

2017 ◽  
Vol 11 (1) ◽  
Author(s):  
Mauro Turrin ◽  
Sergio Martinelli
Keyword(s):  
Acute Pancreatitis ◽  
Differential Diagnosis ◽  
Ct Scan ◽  
Diagnostic Criteria ◽  
Autoimmune Pancreatitis ◽  
Pancreatic Neoplasms ◽  
Low Frequency ◽  
Systemic Diseases ◽  
Histological Confirmation

Among the rarest causes of acute pancreatitis, there are eosinophilic pancreatitis (EP) and autoimmune pancreatitis (AIP). They share some features, such as the low frequency and the association with systemic disorders. Their diagnostic criteria, mainly clinical and laboratory, are different, but both require histological confirmation in most cases: in fact, pancreas is enlarged at CT scan, with tumor-like appearance.Here we discuss the importance of hypereosinophilia in EP and IgG increase in type 1 AIP (included in IgG-related systemic diseases). Differential diagnosis with pancreatic neoplasms and therapy schedules are discussed as well.

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